抗磷脂抗體與台灣硬皮症患者心肺表現的回溯性分析

Antiphospholipid antibodies and cardiopulmonary manifestations in Taiwanese patients with systemic sclerosis – a retrospective analysis

目的：探討抗磷脂抗體與硬皮症重要器官表現的關係。方法：針對在西元1984年至2012年間於臺中榮民總醫院診斷之硬皮症患者進行回溯性分析。結果：一共發現有342名符合美國風濕病學會1980年診斷條件之硬皮症患者。在有抗磷脂抗體資料的病患中，39名（23.6%）為抗心脂抗體陽性，21名（16.0%）為狼瘡抗凝血因子陽性，15名（11.9%）為抗乙二型醣蛋白抗體陽性。抗心脂抗體陽性與心臟超音波下肺高壓和心包膜積水相關，而狼瘡抗凝血因子陽性則比狼瘡抗凝血因子陰性有較短的存活時間。結論：在硬皮症患者中，有抗心脂抗體的人較容易出現肺高壓和心包膜積水，而有狼瘡抗凝血因子的人較容易有較短的存活時間。

Objective: To determine the association between antiphospholipid antibodies and major organ involvement in patients with systemic sclerosis. Methods: Patients who were diagnosed with systemic sclerosis from 1984 to 2012 in Taichung Veterans General Hospital were retrospectively analyzed. Results: A total of 342 patients who fulfilled the American College of Rheumatology 1980 criteria for the classification of systemic sclerosis were identified. Of the patients with available data on antiphospholipid antibodies, 39 (23.6%) were positive for anticardiolipin antibodies (aCL), 21 (16.0%) were positive for lupus anticoagulants (LA), and 15 (11.9%) were positive for anti-beta2-glycoprotein I antibodies (aβ2GPI). The presence of aCL was significantly associated with echocardiographic evidence of pulmonary hypertension (p=0.019, OR 2.58 [95% CI: 1.15-5.76]) and pericardial effusion (p=0.032, OR 2.51 [95% CI: 1.07-5.90]). Positive LA was associated with a shorter mean survival (207.4 [95% CI: 159.0-255.8] months) than negative LA (316.6 [95% CI: 290.2-343.0] months, p=0.034). Conclusion: In patients with systemic sclerosis, those who were ever positive for aCL were more likely to have pulmonary hypertension and pericardial effusion, and those with positive LA were more likely to have a shorter survival.