Clinical Features and Autoantibodies in 164 Taiwanese Patients with Systemic Sclerosis

台灣地區164位全身性硬化症病人的臨床表徵和自體抗體

10.6313/FJR.2007.21(01).04

賴國隆(Kuo-Lung Lai)；陳得源(Der-Yuan Chen)；藍忠亮(Joung-Liang Lan)；黃文瀚(Wen-Han Hwang)

全身性硬化症 ； 臨床表徵 ； 自體抗體 ； 台灣 ； Systemic sclerosis ； clinical features ； autoantibodies ； Taiwan

Formosan Journal of Rheumatology

21卷1期（2007 / 06 / 01）

22 - 31

英文

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.

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.