Clinical Features and Autoantibodies in 164 Taiwanese Patients with Systemic Sclerosis

Abstract

Objective. Systemic sclerosis (SSc) is an autoimmune disorder with fibrosing process involving skin and internal organs. The epidemiology, clinical features and autoantibodies vary in different racial groups. This study aims to determine the clinical features and autoantibodies in Taiwanese patients with SSc, and to detect the associations of autoantibodies with clinical features. Methods. We reviewed the records in 164 patients who diagnosed as SSc at Taichung Veterans General Hospital from 1991 through 2005. Results. The female-to-male (F/M) ratio was 3:1. The mean age at onset was 45.9 years. Seventy-eight patients had limited SSc (lSSc), 83 had diffuse SSc (dSSc), and 3 had SSc sine scleroderma. There was a high prevalence for esophageal involvement (91.3%) and a low prevalence for renal crisis (2.4%). Patients with dSSc, compared with lSSc, were characterized by a higher prevalence for interstitial lung disease (p＜0.001) and acrolysis (p＜0.05). The prevalence of anti-Scl-70, anticentromere (ACA) and anti-U1- ribonucleoprotein (U1RNP) antibodies was 51.8%, 21.3% and 8.5% respectively. Anti-Scl-70 antibodies were associated with dSSc, digital ischemia, acrolysis and interstitial lung disease (p＜0.001 for all). ACA was associated with female predominance, lSSc, sicca symptoms and telangiectasia. Anti-U1RNP antibodies were associated with younger age at onset. The prevalence of antiphospholipid antibodies was 38.3% in our patients, and was associated with digital gangrene and pulmonary hypertension. Conclusion. In comparison with Caucasian, our patients with SSc are characterized by a reduced F/M ratio, a high prevalence for esophageal involvement, a low prevalence for renal crisis, and an increased prevalence for anti-Scl-70 antibodies.