Serological markers in progressive systemic sclerosis: clinical correlations.
The relation between clinical and serological findings was studied in 75 patients with definite progressive systemic sclerosis (PSS). Antinuclear antibodies were detected by indirect immunofluorescence in 95% of the patients by means of HEp-2 cells. The centromere pattern was observed in 50% of the patients with calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, and telangiectasia (CREST) and only 7% of the group with diffuse disease. Precipitating antibodies to nuclear and cytoplasmic antigens were detected by immunodiffusion in 53% of the patients and identified as anti Scl-70 in 16 (21%). We confirmed that autoantibodies are very frequent in PSS but varied in specificity. A combination of immunodiffusion and indirect immunofluorescence was useful in detecting antibodies characteristic of PSS (i.e., anti-Scl-70 and anticentromere) in 51% of these patients. Anticentromere antibody has a high specificity for CREST and identifies patients with less severe disease (i.e., more prolonged course and less involvement of internal organs). Anti-Scl-70 was associated with a high frequency of lung involvement.
D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis; D000918:Antibody Specificity; D034961:Antigens, Nuclear; D001323:Autoantibodies / Q000032:analysis*; D002503:Centromere / Q000276:immunology; D003593:Cytoplasm / Q000276:immunology; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D005779:Immunodiffusion; D008297:Male; D008875:Middle Aged; D009698:Nucleoproteins / Q000276:immunology; D012595:Scleroderma, Systemic / Q000276:immunology*
D000974:Antibodies, Antinuclear; D034961:Antigens, Nuclear; D001323:Autoantibodies; D009698:Nucleoproteins