Systemic sclerosis with a rapidly progressing course
Nasonova, V.A.; Balabanova, R.M.; Alekberova, Z.S.; Mach, E.S.; Iakovleva, G.I.
Terapevticheskii Arkhiv 53(4): 79-86
1981
ISSN/ISBN: 0040-3660 PMID: 7256648 Document Number: 176499
Clinical and laboratory characteristics of a rapidly progressing variant of systemic scleroderma (group 1) are compared with those in a, respectively, selected group of patients (group 2) showing a slow progress of the disease. The rapidly progressing variant was characterized by systemic pathology of the skin which became manifest as early as in the first months of the disease. Evience of this was development of a hard edema with a rapid transition to the induration, a stable change of the system controlling the speed of the synthesis and secretion of collagens, and well as microcirculatory disturbances, such as lowering of the effective cutaneous circulation and an increase of the permeability of the capillaries for protein. Of the visceral symptoms renal pathology was the most frequent and developed earlier in the patients of the group; this pathology was observed in 13 of 15 patients of that group, in 4 of whom it was followed by development of true sclerodermal kidney. Along with this the patients showed a myocardial pathology of the myocardosis type with transition to cardiosclerosis which led to a pronounced hemodynamic deterioration. In group 1 patients, the immune response to connective tissue antigens was more marked than in group 2 patients. Prevalence of type II collagen was noted. Other immunological tests revealed no substantial differences between both variants of the disease, possibly due to the predomination of the sclerotic process over the inflammatory one. The rapidly progressing systemic scleroderma should be regarded as a specific variant of the disease.