Capillaroscopy of the nail bed which diseases. Comparative assessment of the state of the microcirculatory bed of the nail bed and bulbar conjunctiva in patients with systemic scleroderma with various variants of the clinical course. Determination of resistance to
In rheumatology, microcirculation (MC) is studied as part of the “inflammation” problem, and MC disorder is considered as one of the main mechanisms for the development of rheumatic inflammation. The general regularity is violations of hemostasis, rheology and tissue blood flow, changes in the state of intravascular cellular and humoral factors of MC, permeability and transcapillary exchange, biomechanical and other properties of microvessels (Guseva N.G., 1986). However, despite the apparent nonspecificity of vascular changes, with a number of nosological forms, changes in the microvasculature are quite peculiar. Their degree and nature help to conduct differential diagnostics, to more accurately judge the activity of the pathological process, its severity, evaluate the prognosis and effectiveness of treatment of the disease.
Systemic scleroderma (SJS) is a chronic autoimmune disease of connective tissue characterized by generalized vascular damage, progressive fibrosis of the skin and internal organs (Guseva N.G., 1993; Alekperov R.T. et al., 2002; Clements PJ, Furst DE, 2003 ) Key links in pathogenesis
SSDs are cascading microcirculatory disorders: destruction of microvessel endothelium, reduction of capillary basement membranes, minimal proliferation of smooth muscle cells with collagen overproduction (type III) and an increased tendency to vasoconstriction; intravascular changes, thickening of the vascular walls with narrowing of the lumen, which is clinically manifested by generalized Raynaud's syndrome and causes the reduction of the microcirculatory bed characteristic of SSD (LeRoy EC, 1996; Guseva N.G., 2000; 2002; Guseva N.G. et al. 2003; Kahaleh M.V., 2004 and; Kuwana M.et al. 2004).
It is the vessels that are the scene of the development of the pathological process (Guseva N.G., 2003). MC disorders develop in the early stages of the disease (Maricq H.R. et al., 1980; Maricq H.R., 1986; Scheja A. et al., 1996) and precede fibrotic changes in the skin and internal organs (Kahaleh M., 1990 a, b) Changes in MC during SSD are detected in various organs and tissues (Kaye S.A. et al., 1994; Rivolta R. et al., 1996). These changes lead to functional failure of the affected organs due to ischemia and stimulation of fibroplastic processes (Maricq H.R. et al., 1983; Denton C.P. et al., 1996; Rajkumar VS., 1999;
Kahaleh M.V., 2004a, b; Del Rosso A. et al., 2005). However, the genesis of sclerodermic vasculopathy, the intimate mechanisms of damage to the vessels of the microvasculature are not well understood and are being actively studied at present (Guseva N.G.
et al., 2003).
The clinical criteria used for differential diagnosis of primary and secondary Raynaud's syndrome are unreliable, and therefore nail capillaroscopy is of greater diagnostic value (Houtman PM et al., 1986; Nasonov E.L., 1994; Blockmans D. et al., 1996; Anders H. J. et al., 2001). It allows using the presence of capillary changes characteristic of SJS in the differential diagnosis of SJS with other connective tissue diseases in the early stages of their development and Raynaud's disease (Sato S. et al., 1993; Alekperov R.T. et al., 1998; Guseva N. G., 2000a, b; Cutolo M. et al., 2003; Nagy Z., Czirjak L., 2004).
The results of several studies suggest that biomicroscopy of the bulbar conjunctiva (BMBC) reflects the state of microcirculation of the brain and the body as a whole (Braun GS, 1999; Popik G.S. et al., 1999) and can be used to assess systemic disorders of the MC .
Comparison of the data of these two methods in patients with SJS was practically not performed.
The purpose of the study was to evaluate and compare the nature and severity of intravital morphological changes in the microvessels of the nail bed and bulbar conjunctiva in patients with SJS with various variants of the clinical course.
OBJECT AND RESEARCH METHODS
The study included 29 patients with SJS (26 women and 3 men) aged 20 to 57 years (mean age 43.5 ± 1.6 years) who were hospitalized in the City Rheumatology Center at the Central City Clinical Hospital Kiev in 2000-2004 The duration of the disease is from 2 to 27 years (on average - 8.6 ± 1.1 years). All patients showed a characteristic polysyndromic picture of the course of SJS, which corresponded to the main diagnostic criteria recommended for clinical use by the Association of Rheumatologists of Ukraine (Kovalenko V.N., Shuba N.M. (ed.), 2004). A limited diagnosis was diagnosed in 21 (72.4%) patients, and a diffuse form of the disease in 8 (27.6%). Chronic course of the disease was detected in 24 (82.8%) patients, subacute - in 5 (17.2%). All patients were diagnosed with stage II (generalized) disease. In 25 (86.2%) of the examined, the activity of SJS corresponded to I, and in 4 (13.8%) - to II degree.
In all patients, lesions of the skin and peripheral vessels were revealed - Raynaud's syndrome (stage I according to G.L. Ratner and G.E. Slutsker (1989) - in 2 (6.9%), II - in 18 (62.1%) , III - in 7 (24.1%), IV - in 2 (6.9%) patients). Damage to the musculoskeletal system (arthralgia, recurrent polyarthritis)
diagnosed in 22 (75.9%) patients, heart (diffuse cardiosclerosis, according to the clinic, ECG, Echocardiography) - in 21 (72.4%), lung (bilateral, mainly basal, pneumosclerosis according to X-ray examination), - 19 (65.5%), esophagus (esophagitis) - in 15 (51.7%) patients. Less commonly, renal lesions (chronic nephropathy in 4 (13.8%)) and peripheral nervous system (neuropathy in 2 (6.9%) patients) were determined. The control group (CG) included 15 practically healthy individuals, comparable in age and sex with patients in the main group.
Dermal count was determined as the sum of the scores when assessing skin tightness in 17 anatomical regions (Clements P. et al., 1995; Akesson A. et al. 2003).
Structural changes in the capillaries of the nail bed were evaluated by the results of wide-field capillaroscopy. We investigated the condition of the MC of the nail bed of each finger of both hands in all patients, starting from the nail bed of the fifth finger (taking into account its least trauma). The study was carried out after adaptation to room temperature (20-22 ° C) for 15 min using a MBS-10 stereoscopic microscope and an M-70 A capillaroscope with an increase of 3.3-100.8 times. When characterizing the capillaroscopic picture, the following indicators were evaluated: the presence of enlarged and giant capillaries, hemorrhages, branched / bushy capillaries, the severity of the disorganization of the capillary network, the number of functioning capillaries. Changes in the rate of capillary blood flow (accelerated, delayed, stasis), as well as its nature (homogeneous, granular, homogeneous granular) were evaluated. Changes in these indicators were taken into account according to the classification of M. Cutolo et al. (2000), namely:
An early form of MC changes: a small number of giant capillaries and hemorrhages, a relatively well-preserved arrangement of capillaries, a normal number of functioning capillaries;
The active form of MC changes: numerous giant capillaries, numerous hemorrhages, moderate disorganization of capillary architectonics, a moderate decrease in the number of functioning capillaries with avascular fields, the absence or small number of branched capillaries;
Late form of MC changes: uneven expansion of capillaries, insignificant number or absence of giant capillaries, lack of hemorrhages, significant decrease in the number of functioning capillaries with large avascular fields, pronounced disorganization of the capillary network, numerous branched / bushy capillaries.
The condition of the MC of the bulbar conjunctiva was studied using a slit lamp from Zeiss SL 160 (Germany) with an increase of 5-32 times. The diameter of microvessels, the distance between them and the number of vessels per unit area were determined using an object micrometer and a calibration grid. Received
microcirculation indicators that were obtained by morphometric analysis were processed on a scaling RT scale. et al. 1977), which allows us to characterize the qualitative and quantitative changes in the microvasculature. Qualitative indicators included: vascular changes — the ratio of the diameters of arterioles and corresponding venules, gauge unevenness, meander sinuosity, venular sacculations, microaneurysms, glomeruli, reticular structure of blood vessels, changes in the number of functioning capillaries, arterio-venular anastomoses; intratumoral - sludge phenomenon and microthrombi; extravascular - hemorrhages and perivascular edema. Quantitative changes were evaluated by calculating partial (vascular conjunctival index - CI, extravascular CI, intravascular CI) and total CI ^ alai RT. et al., 1977).
Statistical processing of the obtained results was carried out using the statistical software packages “Microsoft ExceI”, “Biostatistics” using the student criterion. Differences were considered significant at p
In the table. 1 presents the clinical characteristics of patients
Table I
Clinical characteristics of patients with SJS
| Index | All patients with SJS (n \u003d 29) | Limited Ssd (n \u003d 21) | Diffuse SSD (n \u003d 8) |
| Age years | 43.5 ± 1.6 (20-57) | 43.0 ± 1.2 (20-57) | 45.6 ± 1.5 (34-56) |
| Gender (female / male) | 26/3 | 20/1 | 6/2 |
| Duration of the disease, years | 8.6 ± 1.1 (2-27) | 8.5 ± 1.5 (2-27) | 8.6 ± 1.2 (4-20) |
| Raynaud's Syndrome
I stage II stage III stage IV stage |
2 (6,9%)
18 (62,1%) 7 (24,1%) 2 (6,9%) |
1 (4,8%)
13 (61,9%) 5 (23,8%) 2 (9,5%) |
1 (12,5%) 5 (62,5%)
2 (25%) 0 |
RESULTS AND ITS DISCUSSION
According to the capillaroscopic characteristic, among the examined patients with SJS, the active form patients prevailed - 21 (72.4%), an early form of MC changes was revealed in (10.3%) patients, and late form in 5 (17.3%) patients. A similar distribution of patients according to the capillaroscopic picture (predominance of patients with an active form) was determined by other authors (Ushiyama O. et al., 2003; Del Rosso A. et al., 2005). In 3 patients with an early form of MC changes, a limited SDS was established. The detection rate of the active form did not significantly differ in patients with diffuse SJS and limited SJS (71.4 and 62.5%, respectively; p\u003e 0.05), and the late form was significantly higher in patients with diffuse SJS - 37.5% versus 9 , 5% in patients with limited (p
The skin count in patients with diffuse SJS was higher than in patients with limited SJS (26.5 ± 2.1 and 9.5 ± 1.7 points, respectively; p
When wide-field capillaroscopy of the nail bed revealed quantitative and qualitative violations of the MC in SSD. The main phenomena
pathological reactions are set at the level of capillaries. SJS patients are characterized by a change in the color and degree of transparency of the capillaroscopic background, which may be due to an increase in the permeability of the capillary membrane to blood plasma proteins. Permeability disturbance was repeatedly observed in SSD (Cervini C., Grassi W., 1996; Guseva N.G. et al.,
2003).
In systemic diseases of the connective tissue, various changes in the size, shape of capillaries and capillary loops were determined, which reflected the processes of capillary destruction and neoangiogenesis (Alekperov R.T. et al., 1998). In the SSD patients examined by us, changes in the size of capillaries and capillary loops were manifested mainly by an increase in the caliber of capillaries and the diameter of capillary loops. All SSD patients showed pronounced polymorphism of capillaries, which were visualized by points, commas, spiraling. In 24 (82.7%) patients, the presence of giant capillaries was detected, which were not determined in patients with CG (p
Possibly, changes in the capillaroscopic picture in SJS are due, in addition to the development of precapillary resistance associated with damage to endothelial cells and structural changes in the arteries of the fingers (Pearson J.D.
1991), tissue squeezing as a result of swelling of the dermis and sclerosis characteristic of this disease (Ushiyama O. et al., 2003).
Thus, during capillaroscopy of the nail bed in patients with SJS, specific changes (giant capillaries, avascular fields, bushy capillaries) and nonspecific changes (change in the color and transparency of the capillaroscopic background, capillary polymorphism, slowing of blood flow with a change in its nature) were revealed. Similar changes in MC according to the data of wide-field capillaroscopy during SJS were determined by other authors (Alekperov R.T. et al., 1998; Bukhari M. et al., 2000; Del Rosso A. et al., 2005).
The results of the study of the state of MC according to BMBC in patients with SJS as a whole indicate its violation in total CI and in all partial CI compared with patients with CG (p
table 2
Quantitative indicators of the state of capillary blood flow of the bulbar conjunctiva (M ± m) in patients with SJS, depending
from a variant of the clinical course
| Index | KG
(n \u003d 15) |
Sick SSD
(n \u003d 29) |
lSD 1 (n \u003d 21) | DSSD 2 (n \u003d 8) |
| Vascular conjunctival index, points | 2.8 ± 0.2 | 10.9 ± 0.7 ** | 9.9 ± 0.8 ** | 13.6 ± 1.0 ** # |
| Extravascular conjunctival index, points | 0.15 ± 0.1 | 1.1 ± 0.1 ** | 1.0 ± 0.2 ** | 1.1 ± 0.3 ** |
| Intravascular conjunctival index, points | 0.6 ± 0.1 | 4.2 ± 0.3 ** | 3.6 ± 0.3 ** | 5.75 ± 0.5 ** ## |
| General
conjunctival index, points |
3.6 ± 0.3 | 16.2 ± 1.0 ** | 14.6 ± 1.1 ** | 20.4 ± 1.6 ** # |
1 lSSD - limited SSD; 2 DSSD - diffuse SSS; *R
In patients with a diffuse form of SJS compared with the limited form, a significant increase in vascular CI was detected (p
Vascular disturbances of the capillary bed during SJS were manifested by gauge unevenness, meander tortuosity, a decrease in the number of functioning capillaries per unit area of \u200b\u200bthe conjunctiva, arterio-spasm, venous dilatation, the presence of aneurysms, venular sacculations, and arterio-venular anastomoses. The number of functioning capillaries is an indicator characterizing the state of capillary blood flow (Chernukh A.M. et al., 1984). A decrease in the number of functioning capillaries, the development of anastomoses indicates insufficient blood supply to the tissue, possible development of tissue ischemia and is an unfavorable factor in the course of the disease. The appearance of anastomoses indicates the presence of obstacles to the blood flow through the capillaries as a result of spasm of microvessels or increased aggregation of red blood cells or perivascular edema. Than
the more pronounced the network of anastomoses, the worse the blood supply to the tissue and the ischemia is aggravated. Extravascular changes were manifested by the presence of hemorrhages and, to a lesser extent, peri-vascular edema. Intravascular changes were characterized by a slowdown in blood flow with the development of stasis and the presence of a sludge phenomenon.
Our results indicate an increase in total CI in patients with SJS, mainly due to the intravascular and extravascular components, vascular changes are less pronounced. In patients with SJS as a whole, a close correlation of the skin count with intravascular CI (g \u003d 0.35), extravascular CI (g \u003d 0.44), vascular CI (g \u003d 0.46) and total CI (g \u003d 0.49; all R
It should be noted that in the available literature, studies on MC based on BMBC data for SJS are few (Popik G.S. et al., 1999) and are devoted to the study of microcirculatory changes in the conjunctiva in children with SJS with digestive organs.
When comparing the integral indicators of the MC status of different vascular pools, a close relationship was found between the severity of changes in the capillaroscopic picture (1 - early, 2 - active, 3 - late) with KI values, primarily intravascular KI (g \u003d 0.52), vascular KI (g \u003d 0.60), as well as extra-vascular CI (g \u003d 0.43) and total CI (g \u003d 0.66; all p
FINDINGS
1. Patients with a diffuse form of SJS are distinguished from patients with a limited form by a greater severity of disorders of the nail bed MC and intravascular and vascular changes in the MC of the bulbar conjunctiva according to the corresponding CI.
2. In patients with SJS, the severity of changes in the capillaroscopic picture is closely correlated with the severity of disorders of the intravascular and vascular units of the MC in BMBC.
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ABSTRACT Complete withdrawal of therapy in patients with proliferative lupus nephritis: long-term follow-up
Moroni G., GaIIeIIiB., QuagIini S., Banfi G., RivoIta E.,
Messa P., PonticeIIi C. (2006)
The fuII canceIIation of therapy in patients with profiIirative Iupus-nephrite: the Iongterm investigation. NephroI. DiaI. TranspIant., Feb. 2.
The unresolved question is whether the complete abolition of glucocorticoid and cytostatic therapy in patients with proliferative lupus nephritis is possible.
Objects and methods. The study included 32 patients with systemic lupus erythematosus (SLE), lupus nephritis, who had previously achieved complete remission, which gradually discontinued all therapy.
Results. After discontinuation of therapy (on average, 38 months after a kidney biopsy), 24 patients remained in remission, 8 patients had proteinuria of 1.05 g / day, while maintaining normal renal function. All patients were monitored for 116-230 months.
15 patients (group 1) were in complete remission. The remaining 17 (group 2) developed an exacerbation of SLE (on average over 34 months), and therefore there was a need to return to the previous therapy. The only difference between these groups was the duration of therapy until it was canceled (57 months in the 1st group and 30 months in the 2nd) and remission (24 and 12 months, respectively) until the therapy was canceled. Subsequently, 12 patients of the 1st group were in complete remission, in 2 - moderate
INFORMATSIYA
proteinuria, 1 - died. In the 2nd group, 1 patient died, remission was noted in 14, moderate proteinuria in 1 and blood creatinine level doubled in 1.
Conclusion. The authors concluded that when complete remission is achieved, nonpharmacological management of patients with lupus nephritis is possible. With a relapse of the disease, resumption of therapy is necessary. The longer the duration of therapy and the period of remission before drug withdrawal, the lower the risk of relapse. The frequency of detection of infections and allergic reactions in patients
with ankylosing spondylitis ZochIing J., BohI-BuhIer M.H. et aI. (200B)
Frequency of infection and aIIergic reactions in patients with spondiIoarthritis. CIin. RheumatoI., 32: 1-11.
Ankylosing spondylitis (AS) is linked to HLA-B27, an antigen of the first class of the main histocompatibility complex, which can affect the physiological immune response. When comparing the frequency of detection of infections and allergic reactions in patients with AS and patients with herniated discs, it was found that over 1 year in patients with AS, episodes of infection were noted in 65.5%, while in the comparison group only in 25.5% of patients (p \u003d 0.0001). Infections of the respiratory tract, gastrointestinal tract and genitourinary system were most often detected. Allergic reactions also occurred more often in patients with AS. This can be explained by both the pathogenetic mechanisms of the disease itself and the influence of basic therapy.
PORVNYALNA ASSESSMENT TO THE CAMP OF THE MICROCIRCULATOR BED OF THE NIGTEVA LODGE AND THE BOULEVARD CON'YUNCTIVITY IN BORIES ON THE SYSTEM SCLEroderma WITH RISE VARIANTS OF THE CLINICAL PERIOD
T.O. Kovganich
39 illnesses were secured for systemic scleroderma (SSD) of vіk vіd 20 to 57 rіkіv (middle vіk - 43.5 ± 1.6 roku). Trivial zhvoryuvannya - type 2 to 27 rok_v (middle - 8.6 ± 1.1 fate). Delivered by the return of the call between the men and women of the capillary picture and the results
bіomіkroskіі bulbarnoe kon’yunktivi at the sick on SSD. Specific capillary changes were revealed: giant capillaries - in 82, Y% were ill for SJS, avascular fields - in BB, 2%. The results of the progress will become microcirculatory (MC) for the data on the bioscopic bulbar Ї conjunctivities of the sick on the SSD to report about the collapse ен yak for the inferior conjunctival index (KI), so for the whole KII. The correlated star’s appearance has been revealed, with a slight change in the KI size and the maximum systemic character of the MC failure in the ill. We completed the completion of the bend of the MC of the Nizhty Bed and the Bulbar-No Conjunctivi.
Key words:system sklєrodєrmiya, micro-circulation, capillary, 6 microscopic bulbar-bar conjunctivities.
COMPARATIVE ESTIMATION CONDITION OF MICROCIRCULATION OF NAILFOLD
AND BULBAR CONJUNCTIVA IN PATIENTS WITH SYSTEMIC SCLEROSIS WITH DIFFERENT CLINICAL COURSES
T.A. Kovganych
We observed29 patients with systemic scIerosis (SSc), their age from 20 to BY years (mean age - 43, B ± 1.6). The continuation of
disease is from 2 year to 2Y years (mean disease duration - 8.6 ± 1.1). There were examined the correIations between changes of capiIIaroscopic pictures and resuIts of biomicroscopy of buIbar conjunctiva in patients with SSc. NaiIfoId capiIIary morphoIogy to Iook for capiIIary abnormaIities and specificity for SSC, such as giant Ioops - n 82, Y% patients with SSc, avascuIar areas - n BB, 2%. We reveaIed disorder microcircuIation for resuIts of biomicroscopy of buIbar conjunctiva in totaI conjunctivaI index and particuIar conjunctivaI indexes. We reveaIed correIation of changes of capiIIaroscopic pictures expressed with quantity conjunctiveIy indexes. This process approve systemic damage of microcircuIation in patients with SSc. We pursued comparison changing expressed of microcircuIation of naiIfoId capiIIary and buIbar conjunctiva for the first time.
Key words:systemic sclerosis, microcirculation, nailfold capillaroscopy, biomicroscopy of bulbar conjunctiva.
Capillaroscopy is a non-invasive technique for diagnosing the state of the human capillary system. Based on the results of the examination, a conclusion is made about the state of the microcirculation system of the body. With this method, gums, cuticles of the fingers and similar areas are examined. Capillaroscopy as a diagnostic method is used in many fields, such as endocrinology, dentistry, cardiology, surgery, etc.
The relevance of diagnosis for medicine
The microcirculation taking place in the capillaries is an object of great interest on the part of doctors, since problems with it associated with various diseases appear very, very often, which allows them to be characterized as a common element of disorders in a patient. The reason for this is that capillaries are a way of delivering nutrients and oxygen to tissues, and it is precisely in them that diffusion, phagocytosis, thrombosis, pinocytosis, etc. go. Another system of capillaries is characterized by a quick response to external influences and changes in homeostasis. By registering this reaction, the doctor can get a lot of important information for diagnosing the patient’s condition, which will allow you to evaluate many aspects of therapy, nutrition, measures taken, etc.
Nowadays, medical workers examine and monitor the activity of capillaries using ultrasound Doppler ultrasound (UDG) and LDF (laser Doppler flowmetry) procedures. Despite the fact that these methods provide a lot of information, they still do not allow quantifying the properties of capillary microcirculation. For this, a recently developed capillaroscopy technique is used.
Another important area where this diagnostic method is used is monitoring the patient's condition during surgery. For example, the quality of the course of heart surgery, when cardiopulmonary bypass is used, can be assessed by whether there is enough oxygen to the patient’s tissues at all stages of the operation. Other methods that are currently in use make it impossible to monitor the parameters of processes such as oxygen diffusion, concentration and dissociation of oxyhemoglobin in the capillaries, while they are extremely important for monitoring the progress of the operation and evaluating oxygen therapy after surgery, which is necessary to eliminate consequences of anesthesia, airway obstruction, pain. Using capillaroscopy, the deoxygenation process occurring on segments from the arterial section to the venous section is also monitored.
The principles of capillaroscopy
Capillaroscopy, or as it is also called capillar spectrometry, and the equipment for its implementation are fully suitable for the requirements developed for medical technologies and medical equipment in recent years:
High technology - equipment effectively uses the latest scientific achievements, such as nanotechnology;
Non-invasiveness - the procedure does not violate the body’s natural external barriers, i.e. mucous membranes and skin, blood sampling is not needed, and incoming data are processed in real time;
Important diagnostic significance;
Safety - since the procedure is non-invasive, there is no risk of infection in the body, and there are no contraindications for the procedure;
Innovation - the technology increases the effectiveness of medical care, as it gives the specialist the opportunity to more accurately select the type of therapy and improves the quality of operations, which often can not give alternative methods.
History reference
The interest in microcirculation processes attracted scientists for a very, very long time. The first who could see the capillaries with his own eyes was Anthony van Levenuk, and he did this in the 17th century. Microcirculation mechanisms were studied by such scientists as Werner Shpaltegolts, Marcello Malpigi, Abram Zalmanov, August Krog. The most widely studied capillaries were a group of scientists led by O. Muller, who owned the idea of \u200b\u200busing microscopes to study the state of capillaries, which subsequently developed into the existing capillaroscopy technique, adopted in 1922.
Capillaroscopy method (K) - this is a study of the nail roll or bed of the capillary and vascular circulation using a special device or microscope - a capillaroscope, an available device for fixing a finger and a light source. Using capillaroscopy, it is possible to study the functions of human capillaries under physiological and pathological conditions.
To. Is a safe non-invasive method for the diagnosis and control of the course of rheumatic diseases, in particular, for the early diagnosis of Systemic scleroderma (SJS) and Raynaud's syndrome (SR). Currently, K. is widely used in the diagnosis and treatment of SJS, it is included in the diagnostic criteria, that is, in order to diagnose SJS and SR, this study is mandatory. In addition, it is used to diagnose and monitor treatment for other rheumatic diseases - Systemic lupus erythematosus (SLE), Dermatomyositis, Rheumatoid arthritis (RA).
K. carried out in several stages. First - an examination of the entire nail bed and nail roller. At the same time, they look at the background of the microscopic field, the shape of the capillaries and the properties of the blood flow in them. Then choose one of the fields of view, consider the number of capillaries, study the movement of blood flow. This data is saved as a photograph for further printing and comparison in dynamics.
Today in Kazakhstan, this study is carried out only in the “Medical Center for Joint Diseases” (ICBC). Our rheumatologist Shosaid Makhmudov was trained at the FSBI Research Institute of Rheumatology named after Nasonova (Moscow) under the guidance of Professor Alekberov R.T. and already 2 years engaged in this method. In September 2016, the 7th EULAR (European Anti-Rheumatic League) course was held on the topic: “Capillaroscopy for rheumatic diseases” in the city of Genoa (Italy). Dr. Makhmudov successfully completed training in English, received a certificate and is the only certified specialist in this field in Kazakhstan.
You can go through the study, as well as other studies to diagnose joint diseases and get advice from a qualified specialist-rheumatologist at the ICBC. Here you can choose the right treatment, including genetic engineering biological therapy, combined pulse therapy, the whole range of physiotherapy (Acupuncture, physical therapy, massage, etc.). If necessary, we can offer both inpatient treatment and day care. You can consult (including online) with leading experts of the Institute of Rheumatology (Moscow)
Dear Patients! An early visit to a rheumatologist for joint pain and qualified treatment will prolong the performance of your joints for many years.
A hundred years to your joints without major repairs!
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Scleroderma is a chronic connective tissue disease that affects the skin and some internal organs of the human body. The basis of this ailment is a violation of the properties of the connective tissue skeleton of organs, which leads to sclerotic changes, that is, to the appearance of gross non-functional fibrous fibers ( scar tissue).
Scleroderma is an autoimmune rheumatologic disease. The term “autoimmune” means that the basis of this disease is a violation of the immune system, which for some reason begins to “attack” the cells and tissues of its own body. The consequence of this is an inflammatory reaction that leads to thinning and tightening of the skin, blood vessels, as well as a number of internal organs ( such as the esophagus, lungs, kidneys, stomach, intestines, heart).
Despite the different localization of the lesions, it is impossible to clearly distinguish the forms of scleroderma. Moreover, many prominent researchers believe that both forms of the disease are the result of the same pathological process.
Scleroderma is a pathological condition that significantly complicates the life of patients. This is due, first of all, to the restriction of physical activity, as well as to pains that can occur in some situations. Due to digestive problems, such patients often need a special diet, they are forced to eat often in small portions. Due to degenerative-sclerotic changes in the skin, patients have to constantly monitor the degree of its hydration, as well as to be extremely careful during sports or any other physical activity.
In addition, many patients with scleroderma experience psychological discomfort associated with thoughts about a disease that is chronic and currently incurable. Since this ailment can cause significant changes in appearance, self-esteem and personal image suffer, which leads to various social and personal conflicts.
The psychological support of patients with scleroderma, which should be provided by family members, friends and relatives, is extremely important and allows you to maintain an adequate quality of life.
Interesting Facts
- scleroderma is a Greek term that comes from the words skleros ( solid) and derma ( leather);
- the term "scleroderma" was introduced into medical practice in 1847;
- the first description of the clinical picture of scleroderma was made in the XVII century;
- among autoimmune diseases, scleroderma is one of the most common ailments;
- more than 75% of patients with scleroderma are women;
- the disease can affect adults and children, however, it is mainly registered in the age group of 30 to 50 years.
Causes of Scleroderma
Scleroderma is an acquired chronic disease of connective tissue, the exact cause of which is still unclear. However, thanks to the development of medicine, molecular biology and laboratory diagnostics, it has become possible to identify and study the main pathological mechanisms involved in the development of the disease. Today, there are several theories that describe factors that can cause scleroderma, as well as the mechanism of their effect. It is believed that the following factors may be involved in the development of scleroderma:
- genetic factor;
- inflammatory factor;
- autoimmune process;
- infectious factor;
- environmental factors;
- a number of medications.
Genetic factor
To date, the question of how large the role of genetic factors in the development of scleroderma is still open. The fact that there is a certain genetic predisposition to the disease, which is confirmed by a higher incidence among first-generation relatives, is indisputable, but the mechanisms that can trigger the work of pathological genes are not completely understood.Some studies have revealed that the occurrence of scleroderma is most likely due to a defect in the HLA-9QA1 gene. This gene is responsible for the synthesis of proteins that play a key role in the functioning of the immune system. These proteins, called the Human Leukocyte Antigen ( human leukocyte antigen - HLA), are presented on the surface of a number of cells and serve to ensure that the immune system can distinguish its own biological material from bacteria and viruses and does not attack its cells. Changes in the structure of this gene and, accordingly, in the structure of surface proteins of cells lead to various autoimmune reactions and, according to several studies, are often detected in patients with scleroderma. There are several other genes involved in the regulation of immune processes, the defeat of which can cause the development of this disease. Unfortunately, the studies carried out at the moment reveal rather contradictory data on the involvement of certain genes in the development of the disease.
The main point in the development of scleroderma is damage to the connective tissue, which can occur for many reasons, including due to genetic defects. Damage to the genes responsible for the production of a factor that regulates the growth of connective tissue, as well as a factor that controls the formation of fibroblasts ( cells that synthesize connective tissue), is one of the potential causes of scleroderma.
Changes in the genetic apparatus can be either congenital or acquired. It should be noted that scleroderma, as such, is not inherited, however, people who are closely related to patients have a much higher chance that the disease will develop.
Changes in the structure of genes are called mutations. They arise spontaneously as a result of random permutations of nucleotides ( structural acids encoding genetic information) during division of ordinary ( somatic) and germ cells. In order for the protein encoded by the gene to acquire a pathological structure, it is enough that only one nucleotide is replaced by another. Nevertheless, it should be understood that the human genome has repair mechanisms that successfully eliminate most of the consequences of incorrect replication ( breeding) chromosomes.
Genome mutations can occur under the influence of the following factors:
- ionizing radiation;
- ultraviolet radiation;
- high and low temperatures;
- nitrates;
- pesticides;
- some drugs ( cytostatics);
- nutritional supplements;
- solvents;
- viruses;
- antigens of certain bacteria.
Inflammatory factor
An inflammatory reaction is the factor that, under a certain set of circumstances, can cause the development of scleroderma. This is due to the fact that during the inflammatory reaction, the production of various biologically active substances increases, which can affect tissues and cause functional and structural changes in cells and vessels. Due to this, vascular permeability increases, conditions are created under which immunocompetent cells are “attracted” to the inflammatory focus. All this creates the prerequisites for the activation of fibroblasts ( cells synthesizing connective tissue fibers) As a result, a chronic inflammatory reaction occurs, which, against the background of genetic abnormalities or exposure to a number of other factors, triggers systemic scleroderma.Autoimmune process
The autoimmune process is a consequence of a disruption in the normal functioning of the immune system and is a pathological immune response directed against the body’s own tissues.The occurrence of an autoimmune process may be associated with the following factors:
- Genetic abnormalities. As mentioned above, with genetic pathologies, the synthesis of a normal surface protein can be disrupted, which leads to a situation in which immune cells do not recognize their own tissues and attack them.
- Pathogenic agents. Some pathogenic agents have antigens that are similar in structure to human proteins. This allows them to protect themselves from immunity, however, with the development of the response, this creates the prerequisites for a parallel attack by the immune cells and antibodies of bacteria and their own tissues.
- Other reasons. Autoimmune processes are extremely complex and can occur under the influence of many reasons.
Infectious factor
For a long time, infectious agents have been considered as a factor that can initiate scleroderma. However, to date, it has not been possible to identify a specific infection, which could act as a provoking factor.Nevertheless, it is believed that some infectious agents are capable of triggering autoimmune reactions by acting on the immune system and thereby provoke the development of systemic scleroderma. In some patients, serum antibodies to protein fragments of human cytomegalovirus were detected, which under certain conditions can interact with normal body tissues. This is due to the fact that the proteins of the virus have the ability to mimicry, that is, they form structures that are similar in structure to the proteins of the human carrier. Due to this mechanism, when the immune response develops, immunocompetent cells attack not only the antigens of the infectious agent, but also their own tissues.
Thus, antibodies to cytomegaly virus fragments have the following effects on the body:
- Fibroblast activation. Fibroblasts are body cells that synthesize connective tissue fibers. They are activated by anti-cytomegalovirus antibodies, as well as under conditions of hypoxia, tissue damage, and inflammatory reactions. At the same time, due to excessive synthesis of collagen, which forms a rigid framework, tissues lose their original elasticity.
- Proliferation of vascular smooth muscle. Under the influence of antibodies, the multiplication of vascular smooth muscle cells is stimulated, which leads to a narrowing of their lumen and, consequently, to hypoxia. This is fraught with progressive damage to cells, as well as some increase in the synthesis of connective tissue.
- The formation of a new inner lining of blood vessels. Under the influence of human cytomegalovirus vascular intima ( sheath lining the inner surface of blood vessels) thickens, which leads to a narrowing of its clearance.
Environmental factors
Medical studies have revealed that there are geographical areas in which the incidence of scleroderma is slightly higher than in other areas. A more detailed study of this phenomenon allowed us to suggest that there are certain environmental factors that in one way or another are able to affect the body and initiate systemic scleroderma.Exposure to the following chemicals can trigger scleroderma:
- silicon;
- heavy metals;
- mercury;
- organic solvents;
- vinyl chloride;
- benzene;
- toluene;
- trichlorethylene;
- epoxy resin;
- urea-formaldehyde resin;
- paraffin and silicone ( used for cosmetic breast augmentation).
- L-tryptophan;
- mazindole
- fenfluramine;
- ampepramone.
The influence of environmental factors can stimulate both local and systemic reactions. The pathological process initiated by any factor continues even after the termination of its effect.
It should be noted that exposure to certain chemicals and environmental factors can trigger sclerotic changes in the skin or internal organs, which, however, are not elements of scleroderma. For example, under the influence of alcohol, a skin pathology occurs, which is outwardly similar to scleroderma, but is not.
It should be understood that these factors can cause the development of scleroderma only if there is an appropriate genetic background.
A number of medications
There are a fairly large number of drugs that, under certain conditions, can cause sclerosis of the skin and internal organs.The following drugs can act as a local provoking factor:
- phytomenadione;
- pentazocine;
- heparin.
The following drugs can cause systemic scleroderma:
- bleomycin;
- L-tryptophan;
- carbidopa;
- penicillamine;
- sodium valproate;
- cocaine;
- amphetamine;
- diltiazem.
Symptoms of Scleroderma
In medical practice, there are limited ( localized) scleroderma, lesions in which are limited to the skin, muscles, joints and bones, and a systemic form in which changes occur in the blood vessels, lungs, kidneys, heart, and other internal organs. Scleroderma is manifested by various clinical symptoms, which depend on the localization of the process and, accordingly, on the affected organs. An important factor influencing the manifestations of pathology is the stage of its development and the degree of sclerotic changes.
In the initial stages of scleroderma, the following non-specific symptoms may occur:
- malaise;
- muscle pain.
Further symptoms of scleroderma depend on the localization and spread of sclerotic foci.
In clinical practice, it is customary to distinguish the following forms of the disease:
- Limited scleroderma. With limited scleroderma, individual foci appear in the thickness of the skin, sometimes muscles and bones. Damage to peripheral blood vessels develops. Depending on the shape and type of foci, plaque, linear and spotted scleroderma are distinguished.
- Systemic scleroderma. With systemic scleroderma, the developing foci are not limited to skin lesions and spread to the internal organs, causing a violation of their function.
Skin lesion
In the evolution of limited skin lesions, three successive stages are distinguished:
- Edema. The inflammatory reaction that occurs initially is accompanied by the release of biologically active substances that cause vasodilation, which leads to edema. As a result, the fingers, hands and feet increase in volume, there is some swelling and pastiness ( when pressed for a while, a trace remains), skin folds are smoothed. In some cases, itching develops. Skin color can be bright red or red with a bluish tint. This stage lasts several weeks.
- Seal. At the stage of compaction or sclerotherapy, the normal structure of the connective tissue of the skin is replaced by a pathological one. Because of this, waxy yellow foci of sclerosis appear, surrounded by a bluish-purple growth zone. In the area of \u200b\u200bthese foci, the skin is hard, cold, inseparable from the underlying tissues.
- Atrophy. Atrophy is the final stage of the process. At this phase of the pathology, the skin becomes thinner, loses its color, resembles parchment paper. Due to the defeat of sweat and fat glands, the skin is dry, easily cracking under the influence of any factors. In case of damage to the subcutaneous fatty tissue and muscles, the skin can adjoin directly to the underlying bone structures.
Depending on the distribution and type of sclerotic foci on the surface of the skin, several forms of the disease are distinguished, among which plaque scleroderma is most often found, named because of the shape of the primary focus, which forms plaques on the surface of the skin.
Forms of scleroderma depending on the type of sclerotic foci
| Scleroderma Form | Characteristic | Photo |
| Plaque scleroderma | Plaque scleroderma is the most common form of the disease. Initially, edema foci of up to 1 - 5 cm in size, round, pinkish in color appear on the skin, which gradually increase and spread ( while in the center there is a progressive compaction) In the stage of development of atrophy, the skin becomes thin, its retraction and blanching is observed. In some cases, calcium deposits are formed in the foci. |  |
| Linear scleroderma | Linear scleroderma is more common among children. It is characterized by one focus located on the scalp, which gradually spreads to the forehead and nose. Due to the linear distribution, this focus resembles a scar from a saber strike. When localized in other parts of the body ( rarely) sclerotic changes spread along the nerve trunks. |  |
| White spot disease | With white spot disease, small, up to one centimeter in diameter, roundish and shiny foci of white color appear. These spots are characterized by a dense structure and a raised surface. A reddish growth zone is visible at the border with healthy tissue. Most often, foci are located on the neck, less often - on the body ( shoulders, chest), as well as on the mucous membrane of the oral cavity and genitals. |  |
| Bullous scleroderma | Bullous scleroderma is characterized by the formation of vesicles on the surface of the skin. It is the result of damage to the lymphatic vessels in combination with the release of biologically active substances from eosinophils ( one type of white blood cell) With this form of the disease, other types of sclerotic foci are usually detected. |  |
| Limited scleroderma with unilateral progressive face atrophy | This form of scleroderma can occur both independently and in combination with other forms of the disease. Usually observed in people under the age of 18 - 22 years. Sclerotic changes begin in the eye, zygomatic arch and lower jaw. In this case, the skin atrophy, bypassing the stage of edema and sclerosis. Due to the involvement of the underlying tissues, severe chronic pain occurs. The process is accompanied by hair loss, eyelashes, eyebrows. Unlike other forms of scleroderma, the activity of sweat and fat glands in this type of ailment does not decrease, but, on the contrary, increases. Due to atrophy of muscles and bones, as well as due to nerve damage, the face loses its symmetry, the affected half decreases in size. |  |
In areas of the affected skin, spider veins and calcification sites form. Spider veins ( telangiectasia) most often occur on the face, mucous membranes of the mouth, eyes, genitals, on the chest, on the hands. Subcutaneous calcification occurs when calcium salts are deposited in the thickness of the skin or subcutaneous fat. This process usually occurs in areas prone to frequent injuries ( front surface of forearms, fingertips) Calcium deposits can over time destroy the skin and form ulcers.
Vascular damage
The action of pathogenic mechanisms in scleroderma is not limited only to the skin, but also covers blood vessels. In almost all patients with this disease, vascular damage manifests itself in the form of Raynaud's syndrome - a spasm of peripheral arteries and arterioles under the influence of low temperatures or stress. Usually this phenomenon covers the fingers, but in rare cases it can be detected on the legs, and even on the tongue. Classically, the Raynaud phenomenon is described as a three-phase change in the color of the skin of fingers. With Raynaud's phenomenon, the following phases of skin color change are detected:
- Pale fingers. Under the influence of cold, excessive spasm of arteries and arterioles occurs ( vessels carrying oxygen-rich blood from the heart), which leads to a decrease in blood supply and, accordingly, to tissue ischemia and pallor of the skin. Usually there is a clear border between the pale fingers and the brush of normal color.
- Blue fingers.The developed vasospasm and insufficient intake of nutrients and oxygen lead to the fact that hemoglobin begins to accumulate in the fingers, which gives off oxygen and combines with the carbon dioxide molecule, which makes the skin bluish ( cyanotic) shade.
- Redness of the fingers. After elimination of vasospasm, redness occurs, which is associated with reactive plethora ( the blood rushes into the exhausted and incapable of adequate contraction peripheral vessels).
In addition to changes in skin color with the Raynaud phenomenon, the following symptoms may occur:
- numbness of the fingers;
- tingling in the fingers;
- local discomfort during an attack.
In some cases, with a long-standing disease, the Raynaud phenomenon may be accompanied by the following symptoms:
- poorly healing sores on the fingers;
- dry gangrene of the fingers;
- spontaneous amputation of affected fingers ( finger dying).
Damage to internal organs
With systemic scleroderma, the following organs and organ systems can be affected:- gastrointestinal tract;
- liver;
- lungs;
- a heart;
- kidneys
- bones and muscles;
- genitourinary system.
Damage to the gastrointestinal tract ( Gastrointestinal tract) is one of the most frequent manifestations of systemic scleroderma among patients who do not have Raynaud's phenomenon. The entire digestive system may be affected, but more often there is sclerosis of the oropharynx, esophagus, stomach, large and small intestines, and rectum. Damage to the salivary glands is possible, which leads to impaired swallowing.
Atrophy of smooth muscles is the cornerstone of the defeat of the gastrointestinal tract ( due to vascular and nerve endings) and fibrosis ( replacement of normal connective tissue) All this leads to the fact that there is a violation of peristalsis and transit of the contents of the digestive system. Sclerosis of the intestinal mucosa leads to impaired absorption of nutrients, which is fraught with weight loss and insufficient production of necessary vitamins and minerals.
Damage to the gastrointestinal tract is accompanied by the following symptoms:
- decrease in the volume of the oral cavity;
- dry mouth
- violation of swallowing;
- heartburn;
- involuntary regurgitation of gastric contents;
- narrowing of the esophagus ( manifested by an early feeling of fullness, heaviness behind the sternum, regurgitation of undigested food, weight loss);
- early saturation;
- latent intestinal bleeding;
- vomiting of blood;
Liver
Scleroderma rarely directly affects liver tissue, but in some cases it can act as a factor contributing to the development of primary biliary cirrhosis. Moreover, due to an increase in the level of bilirubin ( the breakdown product of hemoglobin, which is normally processed by the liver and excreted along with feces) jaundice occurs. When sclerosing the bile ducts, colorless feces, jaundice, and itching may occur.
Lungs
Lung damage is the most common cause of death among patients with scleroderma.
Damage to the lungs with scleroderma can be of the following nature:
- Interstitial lung disease. An interstitium is an organ framework that consists of connective tissue, blood and lymph vessels, as well as the tissue surrounding the vessels and nerves. With interstitial lung disease, sclerosis of the capillaries and walls of the alveoli occurs, which greatly complicates gas exchange processes in the lungs. As a result, shortness of breath develops, lung mobility decreases, and tidal volume decreases.
- Pulmonary hypertension.An increase in pressure in the pulmonary artery system is called pulmonary hypertension. Hypertension develops due to a decrease in the internal lumen of the vessels, as well as due to a decrease in the elasticity of the vascular wall, which leads to an increase in blood flow resistance. As a result, the load on the right parts of the heart increases, and progressive hypertrophy of the right ventricle occurs. Due to the increased pressure in the right calving of the heart, venous return decreases, which leads to stagnation of blood in the liver and some other organs of the pulmonary circulation. As a result, shortness of breath, pain behind the sternum, pain in the right hypochondrium, bloating of the jugular veins are observed.
Damage to the heart with scleroderma is quite common, however, clinical manifestations in many cases are absent. It is believed that a clinically pronounced lesion of the heart muscle and pericardial structures in scleroderma indicates an unfavorable course of the disease.
Damage to the heart with scleroderma can be manifested by the following clinical signs:
- pain behind the sternum;
- shortness of breath during exercise;
- feeling of palpitations;
- dizziness
- bloating of the jugular veins;
- heart rhythm disturbance ( arrhythmias).
Kidney
With scleroderma, the renal vessels are affected, which leads to impaired renal function up to the development of acute renal failure. As a result, the amount of blood filtered through the kidneys decreases, and the decay products begin to accumulate in the body, causing various adverse effects.
The disease is usually manifested by arterial hypertension, which occurs in response to a decrease in blood flow in the renal artery system, and which is aimed at maintaining and restoring proper blood supply. In rare cases, the pressure rises slightly, however, in patients in whom the level of blood pressure was normal before the onset of the pathology, it increases quite strongly. This is accompanied by shortness of breath, headaches, the appearance of "flies" or points in front of the eyes, fainting, pulmonary edema, edema of the lower extremities.
It should be noted that the increase in blood pressure against the background of kidney damage in scleroderma is a completely favorable sign, as it indicates the normal function of the heart muscle.
Against the background of renal failure, peripheral edema develops, intoxication with decay products ( headaches, impaired consciousness) In some cases, the formation of effusion in serous bags - in the abdominal cavity ( dropsy, accompanied by an increase in the abdomen in volume and compression of the abdominal organs), in the pleural cavity ( accompanied by respiratory failure) and in the pericardium ( accompanied by heart failure).
Due to impaired renal function, anemia develops, which is accompanied by pallor of the skin and a decrease in resistance to physical activity. Platelet deficiency also occurs, which is accompanied by bleeding of the gums and mucous membranes of the gastrointestinal tract.
Bones and muscles
Many patients note that the earliest symptoms of scleroderma in them were associated with damage to the musculoskeletal system. In most cases, these symptoms are associated with pain during exertion and at rest. Moreover, in some cases, the occurrence of pain in the joints and muscles may be associated with a true inflammatory reaction. The joints of the fingers and hands, as well as the wrist and elbow joints, are most often affected. Due to thinning of the skin and muscle atrophy, it is far from always possible to adequately assess the degree of joint enlargement. Progressive damage to joints and muscles can cause the formation of flexion contractures ( significant limitation of limb mobility in the joint).
Genitourinary System
The defeat of the genitourinary system with scleroderma is accompanied by the following symptoms:
- erectile disfunction;
- bladder sclerosis with a decrease in its volume ( as a result - frequent urination);
- pain during intercourse ( due to a violation of the vaginal glands and vaginal dryness);
- ulceration of the vaginal mucosa;
- decreased sexual desire;
CREST Syndrome
CREST syndrome is an acronym for English terms, which are a list of the most common manifestations of systemic scleroderma.CREST syndrome includes the following pathologies:
- C - Calcinosis - Calcinosis.Calcification is a dense subcutaneous formation, which are deposits of calcium salts. In some cases, they form ulcers in which bacterial agents can infect soft tissues and bones ( osteomyelitis).
- R - Raynauld "s phenomen - Raynaud's phenomenon. Raynaud's phenomenon, as described previously, occurs due to spasm of peripheral vessels and is manifested by a violation of blood circulation in the fingers and toes. It is characterized by a change in the color of the fingers when exposed to low temperatures.
- E - Esophageal dysmotility - violation of the mobility of the esophagus.Due to the defeat of the smooth muscles of the esophagus, the swallowing process is disrupted, involuntary regurgitation occurs in patients, heartburn appears.
- S - Sclerodactyly - sclerodactyly.Sclerodactyly is a pathological condition in which the skin of the fingers is thickened, the subcutaneous tissue is atrophied, and the terminal phalanges are enlarged. Against the background of all these changes, the mobility of the fingers is impaired, the compression of the hand is much more difficult.
- T - Telangiecasia - spider veins.Due to the defeat of the capillaries and other small blood vessels of the skin on the face, upper half of the body and on the mucous membranes of the mouth, eyes and genitals, small red-blue dots appear, similar in shape to asterisks.
Diagnosis of scleroderma
Diagnosis of scleroderma is a dynamic process that requires constant monitoring and monitoring of the patient's condition. This is due to the fact that this ailment is chronic and constantly progressing. This leads to the fact that after a certain period of time, patients have new lesions in the internal organs and the general condition is disturbed. Diagnosis of scleroderma is based on periodic monitoring of patients with the identification of localization of sclerotic foci and determination of the degree of damage to internal organs, as well as a number of additional laboratory tests and procedures. Of greatest value in this process are the clinical manifestations of the disease.
Clinical manifestations
In 1980, the American Rheumatological Association proposed criteria based on clinical symptoms that can be diagnosed with scleroderma when identified. These criteria are conditionally divided into large and small, depending on how often they occur and how specific they are. The presence of just a few combinations of symptoms indicating scleroderma greatly facilitates the differential diagnosis process.Great criteria:
- Skin lesion. Sclerosis of the skin, which spreads from the base to the top of the fingers or toes.
- Sclerodactyly.Thickening of the skin of fingers and hands with limited mobility and expansion of the nail phalanx.
- Scars on the fingertips.The presence of fresh ulcers or scars on the palmar surface of the nail phalanges.
- Basal pulmonary fibrosis ( pneumofibrosis). With basal pneumofibrosis, proliferation of connective tissue in the basal ( lower) departments of the lungs. With scleroderma, the process is symmetrical. It manifests itself as shortness of breath and a decrease in vital volume of the lungs. Identified during a radiological study ( chest x-ray).
The diagnosis of scleroderma requires the presence of one large and at least two small criteria in one patient.
Other symptoms that are not included in these diagnostic criteria are also extremely important in the process of making a diagnosis, as they allow you to determine the type of ailment, its stage, and also indicate the organs involved in the pathological process.
Laboratory tests
Laboratory tests allow you to assess the condition of the whole organism and identify the main metabolic, structural and functional disorders.With scleroderma, the following laboratory tests are performed:
- General blood analysis.A general blood test can detect a decrease in the number of red blood cells ( anemia), which can occur against the background of hidden bleeding or impaired renal function. A decrease in platelet count may also be detected. Sedimentation rate of erythrocytes ( ESR) can be increased ( non-specific sign of inflammatory process), but quite often it is within normal limits. An increase in ESR is a sign of an unfavorable course of the disease.
- Blood chemistry.A biochemical blood test allows you to identify the salts, enzymes, proteins and pigments contained in the blood serum. With scleroderma, the level of muscle enzymes can be increased ( creatine phosphokinase, aldolase) due to the inflammatory process in the muscles. An increase in creatinine, urea, and other nitrogen breakdown products indicates impaired renal function. An increase in bilirubin levels indicates damage to the liver or bile ducts. Increased levels of alanine aminotransferase and aspartate aminotransferase enzymes ( ALT and AST), which usually indicate liver damage, is not informative with scleroderma, since these enzymes can be released from damaged skeletal muscles and the heart.
- General urine analysis.When kidney damage occurs in the general analysis of urine, an elevated level of proteins and oxyproline, as well as a precipitate consisting of red blood cells, are detected.
- CXCL4 Level Definition.CXCL4 is a biologically active substance that is secreted by plasma cells and interferes with the formation of new vessels. With systemic scleroderma, the level of this substance can be increased, which indicates pneumofibrosis and progressive pulmonary hypertension.
- NT-proBNP level definitions.The activation of the cerebral natriuretic peptide occurs due to the cleavage of the NT-proBNP fragment, the level of which in the blood can be determined using a number of laboratory tests. Brain natriuretic peptide is a hormone that is synthesized by the heart muscle in response to excessive stress. Thus, the concentration of NT-proBNP correlates with the severity of pulmonary hypertension.
- C-reactive protein.C-reactive protein is a marker of the acute stage of the inflammatory response. With scleroderma, its level indicates the activity of the disease.
Autoantibodies
Autoantibodies are immunoglobulins that can interact with their own antigens, that is, with their own tissues of the body. The formation of these antibodies is the basis of many autoimmune and rheumatic diseases. An increased level of autoantibodies can be detected long before the development of the clinical picture.The level and type of autoantibodies is important for the initial diagnosis and for the identification of concomitant pathologies, however, it does not allow monitoring the activity of the disease.
With scleroderma, the following types of autoantibodies can be detected:
- Antinuclear antibodies. Antinuclear antibodies ( ANA) are detected in 90% of patients with scleroderma. They are immunoglobulins that are able to attack the contents of cell nuclei. There are several varieties of these antibodies, among which anticentromere antibodies and antibodies to toisomerase I are most often found.
- Antibodies to topoisomerase I. Antibodies to topoisomerase I ( anti-scl-70) are detected in 30% of patients with diffuse scleroderma. Patients with a high level of these antibodies are characterized by an increased risk of developing pneumofibrosis and interstitial lung disease.
- Anticentromeric antibodies. Anticentromeric antibodies are detected in almost half of patients with a limited form of scleroderma. Anticentromeric antibodies are often associated with severe vascular damage and severe Raynaud's phenomenon.
- Anti-RNA polymerase I and III. Antibodies to RNA polymerase I and III are detected in one fifth of patients with diffuse scleroderma. These antibodies correlate with rapidly progressive skin lesions and a high risk of kidney failure. Often detected in people with several types of autoimmune diseases.
- Antiribonucleoprotein antibodies. Antibodies to ribonucleoproteins ( Anti-rnp) are detected in patients with several autoimmune diseases. Indicate skeletal muscle damage and interstitial lung disease.
Radiological research methods
Radiological research methods, that is, methods based on the use of x-ray radiation, allow you to detect changes in the structure of muscles, bones and a number of internal organs.In clinical practice, simple radiography and computed tomography are used. CT scan ( CT) is a more sensitive and modern diagnostic method, however, it involves a slightly higher dose of radiation compared to simple radiography, and its use is far from always justified.
Simple radiography is used to diagnose the following conditions:
- detection of subcutaneous foci of calcification ( which manifest as intense focal areas of dimming);
- monitoring the condition of the nail phalanges of the fingers;
- the exclusion of osteomyelitis with ulcers on the fingers;
- the exclusion of acute intestinal obstruction in violation of intestinal motility;
- identification of symmetric basal pneumofibrosis ( increase in the intensity of the pulmonary pattern in the basal sections).
- fibrosis of the basal parts of the lungs;
- interstitial lung disease.
Electrocardiography
Electrocardiography ( ECG) is a method of routine examination of patients, which allows you to identify structural and functional changes in the heart muscle. Using an ECG, you can detect signs of pulmonary hypertension, arrhythmia, signs of heart failure.Ultrasound procedure
Ultrasound examination allows you to evaluate the structure and function of some internal organs without any risk to the patient.Ultrasound examination allows you to identify the pathology of the following organs:
- liver;
- kidneys
- a heart.
Transthoracic ultrasound imaging ( ultrasound procedure) is a non-invasive examination method in which the device’s sensor is applied to the skin of the chest, and which allows non-invasive determination of the pressure in the pulmonary artery system. An increase in pressure in the pulmonary artery of more than 35 mm Hg indicates pulmonary hypertension and is an indication for catheterization of the right heart ( a more accurate method for determining pressure, which, however, requires the introduction of a special device into the lumen of the pulmonary trunk).
Lung function
Measuring lung vital volume ( and a number of other volumes) is an extremely important examination for systemic scleroderma, as it allows you to evaluate lung function and the degree of their involvement in pathology. Pulmonary function tests must be performed every six months to a year.A decrease in the diffuse capacity of the lungs in combination with a decrease in the vital capacity of the lungs indicates restrictive damage to the lung tissue ( decrease in elasticity and extensibility of the lungs) An isolated decrease in the diffuse capacity of the lungs indicates damage to the pulmonary vessels against the background of their sclerosis or due to pulmonary hypertension.
Gastrointestinal endoscopy
Endoscopy is a diagnostic procedure during which a special flexible endoscope is introduced into the lumen of the esophagus, equipped with an optical system and a lighting system. This examination allows the doctor to assess the condition of the mucous membrane of the esophagus, stomach, and duodenum.With scleroderma, the following changes can be detected:
- reflux of gastric contents into the esophagus due to weakening of the lower sphincter of the esophagus;
- severe esophagitis ( esophageal inflammation);
- fungal infection of the esophagus;
- narrowing of the esophagus;
- barrett's esophagus ( precancerous condition due to irritation of the esophagus mucosa with hydrochloric acid);
- swelling of the esophagus;
- expansion of the blood vessels of the submucosal layer of the stomach.
Capillaroscopy of the nail bed
Capillaroscopy is a non-invasive method of studying the function of capillaries by examining them under a microscope. The fold of the nail bed is usually examined, since at this point the capillaries are close to the surface and are easy to visualize.With scleroderma, fewer capillaries, multiple vasodilation, irregular or inverted capillary loops are detected.
Histological examination
The study of fragments of tissue of the skin and lungs ( biopsy) under the microscope allows you to reliably detect sclerotic changes. A histological examination is resorted to if it is necessary to differentiate systemic sclerosis with other similar diseases if other examination methods are not sufficiently informative.With scleroderma, the following changes are detected:
- tissue fibrosis with excessive deposition of collagen and intercellular substance;
- chronic inflammation with mononuclear cell infiltration ( macrophages and T-lymphocytes);
- proliferation of the inner lining of the vessels, concentric deposits of connective tissue in the vessel wall, narrowing of the lumen of the vessels, thrombosis.
Medication for scleroderma
The therapeutic approach for scleroderma depends primarily on the type of disease, on the degree of damage to internal organs, as well as on the severity of existing clinical manifestations. The current treatment is aimed at eliminating the consequences of sclerotic changes in organs, at preventing possible complications, as well as at eliminating a number of disturbing symptoms. Unfortunately, a complete cure for this ailment is not yet possible.
Scleroderma treatment is carried out using the following groups of drugs:
- enzyme preparations;
- vasodilator drugs;
- drugs that suppress the immune system;
- anti-inflammatory drugs;
- chelation therapy.
Enzyme treatment
Enzyme preparations are capable of cleaving connective tissue fibers that form in the foci of sclerosis. These funds can be administered systemically in the form of intramuscular injections or locally by electrophoresis. The course of treatment depends on the severity of the disease and can last from 5 to 7 days to two to three weeks.The following drugs are possible:
- longidase;
- ronidase;
- lidase;
- trypsin;
- chymotrypsin.
Vasodilator treatment
The use of vasodilating drugs eliminates spasm of blood vessels, which has a beneficial effect on blood circulation in peripheral tissues and in internal organs. Thanks to this, it is possible to reduce the severity of the Raynaud phenomenon, pulmonary hypertension and kidney damage. In addition, vasodilation can reduce the load on the heart muscle.In the treatment of scleroderma, the following vasodilator drugs are used:
- Calcium channel blockers.Calcium channel blockers reduce spasm of vascular smooth muscle by reducing the intake of calcium, a mineral that is necessary for the normal contraction of muscle fibers. Verapamil tablets are used at a dose of 40 - 80 mg 3-4 times a day or nifedipine tablets at a dose of 10 - 20 mg 2 times a day.
- Phosphodiesterase InhibitorsPhosphodiesterase is an enzyme that is involved in the synthesis of a number of substances that can cause vascular contraction. These drugs have the greatest effect on the vessels of the pulmonary circulation, so they are used for pulmonary hypertension. Pentoxifylline is usually used at a dose of 600 mg per day.
Immune Suppressant Treatment
Since autoimmune processes that occur due to a malfunction of the body's immune system play an important role in the development of scleroderma, there is a need to use drugs that suppress the immune system.The following drugs are used to suppress immunity:
- Cyclophosphamide is a drug that is widely used to treat many autoimmune diseases. It reduces the intensity of pulmonary fibrosis, slows down the formation of foci of sclerosis in the tissues of internal organs and skin.
- Methotrexate inhibits the division of immune blood cells, due to which it has a pronounced immunomodulating effect. Studies show its high efficiency in lesions of the skin and lungs, as well as inflammation of the muscles and joints.
- Cyclosporin inhibits the activity of T-lymphocytes, which play a key role in the development of diffuse scleroderma.
- Azathioprine inhibits the metabolism of nucleic acids. Due to this, it blocks the process of cell division and reduces the intensity of the immune response.
- Rituximab is a drug that specifically blocks the B-lymphocytes responsible for the synthesis of antibodies.
It should be borne in mind that these drugs have a large number of side effects ( toxic effects on the kidneys, bone marrow, heart, liver, reduce resistance to infections), therefore, they should only be appointed by a competent specialist. Incorrect use of immunosuppressants can cause many serious complications.
Anti-inflammatory drug treatment
Treatment with non-steroidal anti-inflammatory drugs can reduce the inflammatory response and its accompanying symptoms ( swelling, pain, joint damage).Nonsteroidal anti-inflammatory drugs
| A drug | Mechanism of action | Mode of application |
| Diclofenac | It blocks the enzyme cyclooxygenase, which is involved in the cleavage of arachidonic acid in the focus of inflammation to biologically active substances that have a pro-inflammatory effect. A decrease in the level of these substances eliminates the inflammatory reaction, swelling and pain. | Inside, 75-150 mg in several doses, after a meal with plenty of water. |
| Ibuprofen | Inside, 800 mg 3 times a day after meals, with plenty of water. | |
| Meloxicam | Inside at a dose of 7.5 - 15 mg once a day after meals. | |
| Celecoxib | Inside, in a daily dose of 200 mg in two divided doses. |
It should be borne in mind that most non-steroidal anti-inflammatory drugs negatively affect the gastric mucosa, and if the intake regimen is not followed, they can provoke gastritis and ulcer. For this reason, they are often prescribed with drugs that protect the stomach ( omeprazole, ranitidine, famotidine).
With the ineffectiveness of non-steroidal anti-inflammatory drugs, glucocorticoids - steroid hormones ( dexamethasone, prednisone), which have great anti-inflammatory activity and are able to suppress immunity. The dosage of these drugs is determined strictly individually, since due to the effect on the whole body and due to a number of side effects, their incorrect use can have adverse consequences.
Chelation therapy
Chelation therapy is aimed at eliminating certain substances from the body by binding them to specific drugs. In scleroderma, D-penicillamine is used, which is able to remove copper from the body. Due to this, it is possible to reduce the rate of formation of fibrous tissue and reduce the intensity of the inflammatory reaction ( copper is necessary for the normal functioning of cells synthesizing connective tissue fibers).Hormones and antibiotics for scleroderma
Nutrition for scleroderma ( diet)
Scleroderma does not apply to diseases in which any specific diet is necessary. Nevertheless, proper nutrition can reduce discomfort and alleviate the general condition of the patient.- with damage to the esophagus, solid food should be avoided;
- with bowel damage, it is necessary to eat foods rich in plant fibers;
- a large amount of vitamins and minerals should be consumed, since their absorption in the intestine is often impaired;
- you need to eat enough calories;
- high doses of vitamin C should be avoided ( more than 1000 mg / day), since it stimulates the formation of connective tissue;
- in case of kidney damage, it is advisable to reduce the amount of salt and water supplied with food.
Is bed rest necessary for scleroderma?
The need for bed rest with scleroderma arises only with severe damage to vital internal organs ( heart, lungs, kidneys), when any physical activity can provoke critical complications. In other cases, there is no need for bed rest, and, moreover, a decrease in physical activity is highly not recommended. This is due to the fact that in the absence of movements, atrophy of the skin and muscles develops faster, and the joints lose their mobility.Patients with scleroderma should pay special attention to the temperature regime in which they are, since cold air can provoke a spasm of the vessels of the limbs ( raynaud's phenomenon), which can have many adverse effects.
Traditional methods of treating scleroderma
Alternative methods of treating scleroderma can alleviate the course of the disease, are able to eliminate some external manifestations, and contribute to a slower development of the disease. However, the use of traditional medicine methods without the use of pharmacological drugs may not be effective enough and lead to various complications.The following folk methods are used to treat scleroderma:
- Aloe juice. A compress moistened in aloe juice is applied to previously steamed skin. The procedure should be repeated once every few days. This tool allows you to slightly reduce the stiffness of the skin and the spread rate of sclerotic foci.
- Wormwood ointment. Powdered wormwood grass is mixed with fat ( better with sterile petroleum jelly) in equal proportions. The resulting ointment must be treated with scleroderma foci 2 to 3 times a day for one to two weeks.
- Horsetail infusion. A tablespoon of a mixture of horsetail, knotweed and medunica should be poured with 200 ml of water and kept in a water bath for a quarter of an hour. After the broth has cooled, it should be filtered and drunk 50 ml before meals.
- Broth grass meadowsweet. A tablespoon of grass meadowsweet need to pour two glasses of water and boil for 7 - 15 minutes. The resulting broth must be insisted for several hours, after which it can be drunk 100 ml twice a day. Reduces pain in joints and foci of inflammation.
- Hypericum infusion. Three tablespoons of the crushed mixture of St. John's wort, sweet clover, raspberry, plantain and mint should be poured with 5 cups of boiling water and insisted for 6 - 8 hours, then strain. The resulting infusion should be drunk 50 - 100 ml per day for 2 months.
Answers to frequently asked questions
Can I get scleroderma?
It is impossible to become infected with scleroderma, since this pathology is a consequence of a violation of the normal functioning of the immune system and cells synthesizing connective tissue fibers. Despite the fact that the infection can act as a provoking factor, to date, no specific pathogenic agents have been identified that can cause scleroderma.Scleroderma is a chronic inflammatory disease of an autoimmune nature that affects the connective tissue skeleton of the skin and internal organs. The exact cause of the occurrence of scleroderma is unknown, but it is assumed that the underlying underlying ailment is a genetic predisposition that is activated by some external or internal factors.
The genetic predisposition is based on a defect in the genes responsible for the formation of structures necessary for the recognition of cells and tissues in one's own system - another's during an immune response. As a result of changes in the structure of genes, a protein is formed that is unknown to the human immune system, which leads to the fact that immune cells begin to attack their own tissues. This leads to the development of inflammation and to excessive release of factors that stimulate the formation of connective tissue.
Changes in the structure of the genetic apparatus can occur under the influence of the following factors:
- ionizing radiation;
- ultraviolet radiation;
- nitrates;
- nutritional supplements;
- viruses;
- some drugs.
As evidence of the genetic nature of scleroderma, a large incidence of this disease among close relatives and identical twins is given. However, numerous studies indicate that in most cases a genetic defect appeared only after exposure to some external or internal provoking factor.
The following factors can provoke the development of scleroderma:
- Inflammation.An inflammatory reaction is a factor that, in the presence of a genetic predisposition, can cause the development of systemic scleroderma. This is explained by the production of pro-inflammatory substances that can stimulate fibroblasts - cells that synthesize connective tissue fibers. Against the background of the excessive activity of these cells, dormant genes are activated that begin to synthesize a defective protein. As a result, an immune response to their own tissues is triggered, which stimulates the further development of the disease.
- Autoimmune process.An autoimmune process is a pathological condition in which the human immune system attacks its own tissues.
- Infection.Some infectious agents have an antigenic structure similar to normal tissues in the human body. This leads to the fact that under certain conditions an autoimmune reaction may occur. However, it should be understood that a similar pathological response with scleroderma is possible only if there is a genetic predisposition.
- Environment.Some environmental factors, such as salts of heavy metals, silicon, organic solvents, benzene and toluene, if predisposing factors are present, can cause foci of sclerosis in the skin and internal organs.
- Medications.Some drugs ( bleomycin, cocaine, diltiazem, etc.) can cause activation of defective genes, which can lead to the development of systemic scleroderma.
What do scleroderma patients look like?
The appearance of patients with scleroderma depends on the localization of sclerotic foci, the degree of damage to the skin and limbs, the condition of subcutaneous fatty tissue, muscles and bones, as well as the stage of development of the disease.In the development of scleroderma, one should distinguish between the initial stage, during which the inflammatory process is active, and the later stage, during which the intensity of inflammation decreases and sclerotic and atrophic lesions develop.
Scleroderma can affect the skin, blood vessels, and some internal organs. Depending on the appearance and localization of foci of sclerosis on the skin, several types of this disease are distinguished.
Types of Scleroderma
| Scleroderma Form | Description | Photo |
| Plaque scleroderma | Initially, edematous pinkish, roundish plaques up to 5 cm in size appear on the skin, which gradually increase in size. At the periphery of these foci, a blue-violet border zone is observed. As the foci grow, the skin in the center becomes denser, acquiring a yellowish tint. |  |
| Linear scleroderma | A linear lesion is located on the scalp. Distributes on the skin of the face - forehead, nose, cheeks. The skin in the area of \u200b\u200bthe focus is thin, pale. |  |
| White spot disease | Small shiny foci up to one centimeter in size. They are located on the trunk and mucous membranes of the oral cavity. |  |
| Bullous scleroderma | Bubbles with transparent contents that combine with other forms of sclerotic lesions. |  |
| Limited scleroderma with unilateral facial atrophy | The sclerotic lesion covers the skin around the eyes, in the zygomatic arch and lower jaw. Due to atrophy of the underlying muscles and bones, asymmetry of the face occurs with a decrease in the affected side. |  |
In addition to these specific formations on the skin, with scleroderma, limbs with the formation of ulcers, atrophy of muscles and bones can suffer. Often, foci of sclerosis are found in the neck, face, trunk, and genitals.
Damage to the limbs and trunk with scleroderma
| Type of lesion | Description | Photo |
| Sores on the fingers | Ulcers form on the pads of the nail phalanges of the fingers of the hand ( open defects) of various sizes and shapes. When an infection is attached, suppuration may occur. |  |
| Atrophy of the muscles of the hand | Due to atrophy of the muscle and subcutaneous fatty tissue, bones are easily visible on the surface of the hand, fingers become similar to “bamboo branches” due to visual highlighting of the surface of the joints. Sometimes there is a thickening of the bones of the nail phalanges ( drumsticks). |  |
| Atrophy of the muscles of the lower limb | Atrophy of the muscles and subcutaneous tissue below the level of the knee occurs, so that the lower leg looks thinned. In some cases, the muscles atrophy so much that the skin immediately adjoins the bone. |  |
| Neck skin thickening | The skin in the neck area is thickened and inactive, has a yellowish tint. Due to the decrease in elasticity, the skin cannot be folded. The surface of the skin is dry and cold. |  |
| Thickening of the skin around the mouth | The skin around the mouth is thickened, its elasticity is reduced, due to which the opening of the oral opening is limited. |  |
With scleroderma, the disorders are not limited to the skin only. A lesion of the blood vessels develops, which is manifested by spider veins and Raynaud's phenomenon.
Vascular damage in scleroderma
| Type of vascular lesion | Process stages | Description | Photo |
| Raynaud's phenomenon | Pallor of fingers | Fingers are pale, differ sharply in color from the brush. |  |
| Cyanosis of fingers | Fingers become bluish. |  |
|
| Finger redness | Fingers of red color. With scleroderma, this stage may be absent. |  |
|
| Spider veins | – | Small reddish, branched dots formed by superficial capillaries. They are found on the skin of the chest, shoulders, on the mucous membranes of the mouth, genitals. |  |
The capillary system (capillary bed, terminal bloodstream) is a mediator in the metabolism between blood and tissues. In practice, it is a submacroscopic site of the vascular system.
Among the methods for studying capillaries, capillar microscopy, the study of capillary permeability, and capillary resistance are the most common.
Capillar microscopy
Principle. Capillaries can be observed directly in incident light under a capillary microscope. Observe the filling of capillaries. The filling pattern and the shape or width of the vascular lumen are not always identical, which narrows the applicability of this method.
Methodology for the study of capillaries. Examine the nail roller (brush, foot), the mucous membrane of the lips and the conjunctiva of the eyes. An oily liquid (cedar oil, paraffin oil) is applied to the test site, and glycerin is applied to the lips or conjunctiva. For research, they use a skin capillaroscope, a stereomicroscope, or for observations on the nail roller on the back of the fingers and the back of the hand with an ordinary microscope (illumination at an angle of incidence of a light beam of 45 °).
Assessment of capillary research. On the microscopic picture of the capillaries you can see, if you look from top to bottom: vertically standing capillaries, obliquely extending collecting venules, a horizontally located venous plexus directly below the papillary layer and subcutaneous tissue below the diffuse main background. The shape and location of the vessels, their width and blood circulation in them are taken into account. The detection of blood extravasates is important. To properly take into account the picture requires a lot of experience; the data obtained can only be used to a limited extent.
The study of capillary permeability and resistance
Here, functional samples are distinguished regarding water metabolism, bloodstream, capillary wall, interstitium and lymphatic pathways, as well as functional tests, in which red blood cells pass through the capillary wall by increasing or decreasing blood pressure.
Landis method
Principle. After stagnation of blood in the limb as a result of the application of the cuff under a pressure of 40 mm RT. Art. in healthy individuals there is only a slight loss of fluid and no loss in blood proteins. Functional disorders of the capillaries lead to a significant loss of fluid and protein.
Execution technique. At room temperature, before and after a half-hour stagnation of blood in the shoulder (from a pressure of 40 mm Hg), blood is taken even before the stagnation ceases and the hematocrit value and serum protein content are determined. Based on the obtained 4 numbers, the permeability of the capillary walls for liquid and protein is calculated.
Rating. In healthy individuals with a cuff pressure of 40 mm RT. Art. and a 30-minute duration of the sample, the release of protein is not observed. In pathological cases, protein loss reaches 1 g%. In some cases, errors are inevitable and therefore conclusions can only be drawn in relation to groups of patients.
Cantharidin test
The greater the capillary permeability, the higher the permeability of their wall for substances that, under normal conditions, are retained by the capillary wall.
Research Methodology. Before the study of the capillaries, the patient is placed on the forearm for 12 hours with a 4 x 4 cm cantharidin patch with 0.1% cantharidin. In this case, a bubble forms, it is punctured and its contents are examined. Determine, for example, the content of whey protein, individual fractions of whey protein (by electrophoresis), the content of hexoses associated with proteins.
Rating. If only the capillary permeability is checked, then the content in the patient's serum of the total protein is compared with its content in the vesicle fluid.
Under normal conditions, according to Kiichmeister, the protein content in the serum is 7 g%, in the cystic fluid 5 g%, the difference below 2 g% is pathological.
With age, capillary permeability decreases. Medicines such as calcium, rutin, and esculin lead to a decrease in capillary permeability under the influence of processes that occur on the border between blood and tissue. On the contrary, under the influence of hyaluronidase, tissue permeability increases.
Method for determining capillary pressure by Kiichmeister and Herrnring
The principle of study of capillaries. If we trace under a microscope the loops of capillaries that disappear under the influence of increasing blood pressure, then they can again be found in the deeper layer, and the red blood cells still continue to pass through the loops. The criterion is the moment of termination of blood flow.
Execution technique. They use a capillary microscope connected to a pressure chamber. Observe the behavior of capillary loops before the onset of red blood cell stasis. Pressure values \u200b\u200bare recorded.
Determination of capillary resistance
By capillary resistance is meant the resistance of capillaries to the passage of red blood cells through their wall. The fragility of capillaries is the fragility of the capillary wall, a special form of capillary resistance. In this regard, they also speak of an endothelial symptom.
The following studies of capillaries are of clinical importance for identifying damage to the capillary wall, regardless of their causes (toxicosis, deficiency of vitamins C and P, vascular bleeding).
Rumpel-Leede Phenomenon
Principle. A cuff is used on the shoulder, used to determine blood pressure, and the appearance of blood points on the skin is monitored.
Methodology for the study of capillaries. The pressure in the cuff should be 10-20 mmHg. Art. below systolic pressure; the cuff is left longer than 5 minutes. By removing the cuff, it is observed whether petechiae have appeared, and sometimes their number is counted, for which it is advisable to use a magnifying glass.
The increased number of petechiae in the study of capillaries in women before and during menstruation is still a physiological phenomenon.
Determination of capillary resistance using a suction can
On the skin of the neck (side at the level of the collar) put a glass jar with a diameter of 4 cm with a negative pressure of 50-100 mm RT. Art. (mercury manometer). Using a magnifying glass after 3 minutes, the number of petechiae appearing on the skin is calculated.
Rating. Normally, 2-3 petechiae appear. The appearance of 8 or more petechiae is considered a pathological phenomenon.
With the method of researching capillaries with a suction bath, you can also use a capillary resistometer (according to Kuchmeister and Scharfe). This apparatus consists of a vacuum pump, a vacuum gauge, a vacuum reserve and a glass suction can, into which the set reduced pressure is transmitted by ventilation. At constant pressure (for example, 300 mmHg), the time until the appearance of new 2-3 petechiae is noted. This time serves as an indirect measure of capillary resistance, which in healthy people is 13 ± 2 seconds.
Prepared and edited by: surgeon
