[Sclerodermic pneumofibrosis (clinical-functional characteristics)].
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Biomedical subjects
Publications and source records attributed to N G Guseva.
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Capillaroscopic study of the nail bed of patients with scleroderma systematica (SS) detects avascular regions and neoangiogenesis signs, but the data on the morphological substrate of these changes are lacking. Positive correlation is established between the degree of inflammation and fibroblast number in the skin biopsies on the one hand and expression of the avascular foci, their total surface and sclerodermic processes activity, on the other. Vascular lesions in SS manifested with sclerodermic vasculopathy which in some patients is comorbid with vasculitis of microcirculatory vessels. A wide spectrum of morphological vascular lesions reflects clinical heterogeneity of SS. Vascular lesions is an obligatory component, are manifest at early stages of SS and their transformation reflects the disease evolution.
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The method of optic and electron microscopy was used to examine the skin, muscle and fascia in 8 patients with eosinophilic fasciitis aged from 20 to 57 years with the disease duration from 6 months to 3.5 years. When examining biopsy samples the authors paid special attention to the state of collagenic structure, cellular elements of the skin connective tissue, fascia and intermuscular layers, changes in the cross-striated muscle adjacent to the fascia, composition of cellular infiltrates as well as to the state of vascular microcirculation. It has been shown that with the given disease one can observe exudative-infiltrative processes with alteration of the vascular bed in the fascia and epimysium and the reaction of the microcirculatory bed of the skin and muscle with subsequent outcome into sclerosis at later periods of the disease. But the morphological data allow exclusion of the diffuse nature of microcirculation derangement in eosinophilic fasciitis. Morphogenesis of vascular lesions in this disease differs from such in scleroderma that suggests two different diseases.
A total of 104 patients with scleroderma were examined. Anticardiolipin antibodies were detected in 37.5 per cent of the patients with systemic scleroderma and in 3 per cent of healthy individuals; they were more often detected in 46.8 per cent of the patients with diffuse affections of the skin, atherosclerosis, Raynaud's syndrome accompanied by ulcero-necrotic affections of the skin as compared to patients with restricted affections of the skin (sclerodactylia and focal scleroderma)--29.8 per cent. No significant changes in the frequency of detecting a rheumatic factor, antibodies to Scl-70 were revealed in subgroups of patients with scleroderma, positive and negative anticardiolipin antibodies. Of the greatest interest is a significant difference in levels of C-reactive protein which were high in half of the patients with anticardiolipin antibodies. Anticentromere antibodies were detected twice as more often in patients without anticardiolipin antibodies that corresponded to systemic sclerodermia with minimum involvement of the skin into the pathological process. It is suggested that ulcero-necrotic affection of the skin in systemic sclerodermia is associated with C-reactive protein but it is not of an immunocomplex nature.
The spectrophotometric method was used to determine the activity of beta-glucuronidase (BGU) in the cells (neutrophils, mononuclear cells, thrombocytes) in the blood serum of patients with systemic sclerodermia (SSD). The authors revealed a synchronous change in the activity of BGU in all the cells and in the blood serum, and also a decrease in the activity of the enzyme in the concentration of protein in neutrophils, it being very significant in patients with SSD accompanied by trophic changes. There was a direct relationship between the activity of BGU in the blood serum and the level of C-reactive protein and an inverse one between the activity of BGU and the content of circulating immune complexes in the blood. The level of BGU in the blood serum reflects not only the activity of the inflammatory process but also the markedness of trophic changes in patients with SSD.
The authors studied clinical course, morphological findings, condition of microcirculation in 22 patients with diffuse fasciitis++ in comparison with 20 patients with systemic scleroderma and 20 patients with other similar diseases. Comparing clinico-laboratory data of 62 patients sharp differences were observed between diffuse fasciitis++ and systemic scleroderma. In diffuse fasciitis++ microcirculation disorders were less marked than in systemic scleroderma. According to the above research diffuse eosinophilic fasciitis++ is considered to be an independent nosologic form.
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