The distribution of HLA-A, B, C antigens has been studied in 40 patients with systemic scleroderma and in 200 healthy individuals (all Russians). An increased frequency of the antigens B35 and Cw4 has been discovered in patients, as compared with control. When analysing different clinical and common parameters, lung affection in the systemic scleroderma patients was found to be associated with the antigen A10 (58.9% versus 21% in control, RR = 5.22, EF = 0.476, Pc = 0.0363), the presence of antinuclear antibodies being associated with the antigen B35 (50% versus 17% in control, RR = 4.8, EF = 0.396, Pc = 0.0354). The association with the antigen B8 most commonly mentioned in the literature was characteristic of the patients with an earlier onset of the disease (under 30 years) and those with the rheumatoid factor. The patients having D-penicillamine-induced complications were found to have an increased frequency of the antigen B8, as compared with the alternative group of patients (2P = 0.0430).
HLA-A Antigens/genetics , HLA-B Antigens/genetics , HLA-C Antigens/genetics , Penicillamine/therapeutic use , Scleroderma, Systemic/immunology , Adolescent , Adult , Aged , Female , Gene Frequency , Humans , Male , Middle Aged , Penicillamine/adverse effects , Scleroderma, Systemic/drug therapy , Scleroderma, Systemic/genetics , Scleroderma, Systemic/pathology , USSR
Paraneoplastic Syndromes/diagnosis , Scleroderma, Systemic/diagnosis , Adult , Diagnosis, Differential , Diagnostic Errors , Female , Humans , Male , Middle Aged , Neoplasm Metastasis , Paraneoplastic Syndromes/pathology , Raynaud Disease/diagnosis , Raynaud Disease/pathology , Scleroderma, Systemic/pathology
Dextrans/administration & dosage , Scleroderma, Systemic/drug therapy , Adult , Blood Viscosity/drug effects , Capillary Permeability/drug effects , Chronic Disease , Colloids , Drug Evaluation , Humans , Male , Microcirculation/drug effects , Molecular Weight , Platelet Aggregation/drug effects , Scleroderma, Systemic/physiopathology
Six patients with eosinophilic fasciitis are presented. This syndrome is characterized by indurative swellings of arms and legs, with rapidly progressing difficulties in extending elbows, wrists, and fingers, and often limited motion of shoulders and ankle joints. Pain when contracting muscles, and weakness of proximal muscles and hand grip are common features. The frequent occurrence of localized skin lesions has presented differential diagnostic difficulties to systemic sclerosis and to polymyositis. Visceral involvement and Raynaud's phenomena, however, are absent or mild. Blood eosinophilia, hypergammaglobulinemia, and unspecific signs of inflammation are found. Biopsy of muscle fascia gives characteristic histopathological findings of cell infiltrations (mostly mononuclear cells, frequently eosinophils) and vascular proliferation, in the middle layer of a thickened fascia. Skin changes are prevalent, but not conclusive for the diagnosis, and myositis in some patients might be difficult to distinguish from polymyositis. The importance of the clinical recognition of eosinophilic fasciitis and the inclusion of fascia in diagnostic muscle biopsies, is underlined.
Eosinophils , Fascia , Inflammation/blood , Adult , Diagnosis, Differential , Fascia/pathology , Female , Humans , Inflammation/pathology , Male , Middle Aged , Muscles/enzymology , Muscles/pathology , Myositis/diagnosis , Skin/pathology
Eosinophils , Fascia , Adult , Female , Humans , Inflammation/diagnosis , Male , Middle Aged , Syndrome
