台灣地區164位全身性硬化症病人的臨床表徵和自體抗體

Clinical features and autoantibodies in 164 Taiwanese patients with systemic sclerosis

前言：全身性硬化症是一種侵犯皮膚和內臟器官導致纖維化的自體免疫疾病。其流行病學，臨床表徵和自體抗體於不同種族間亦不相同。本研究的目的在於探討台灣地區全身性硬化症病人的臨床表徵和，自體抗體，並評估自體抗體和臨床表徵之間的關聯性。方法：我們回顧1991年至2005年，於台北榮民總醫院診斷為全身性硬化症，共164位病人。我們就臨床表徵和自體抗體予以分析。結果：女性和男性的比例為3：1。平均發病年齡為45.9歲。侷限型全身性硬化症有78人，廣泛型全身性硬化症有83人，無皮膚硬化的全身性硬化症有3人。食道侵犯的盛行率高（91.3%）而腎危機的盛行率低（2.4%）。相較於侷限型全身性硬化症，廣泛型全身性硬化症病人有較多的間質性肺病（p<0.OO1）和肢端溶解現象（p<0.05）。Anti-Scl-70抗體、anticentromere抗體和anti-U1RNP的抗體的盛行率分別為51.8%、21.3%和8.5%。Anti-Scl-70抗飽和廣泛型全身性硬化症、指端缺血、肢端溶解和間質性肺病有相關性（p皆<0.001）。Anticentromere抗體和女性、侷限型全身性硬化症、乾燥症狀和微血管擴張有相關性。Anti-U1RNP抗體和發病年齡較低有相關性。抗磷脂抗體的盛行率為38.3%；此抗體和指端壞死以及肺高壓相關。結論：和白種人相較，我們的全身性硬化症病人的特徵有：較低的女／男比例，較多的食道侵犯，較少的腎危機，和較高的anti-Scl-70抗體盛行率。

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 (FIM) 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 (9l.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-UI-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.