Jo-1抗體陽性病人之臨床特徵及血清學表現

Clinical and serological features of patients with anti-Jo-1 antibodies

目的：研究Jo-1抗體陽性病人之臨床徵及血清學表現。方法：本研究收錄在一醫學中心自2007年至2010年間測得Jo-1抗體陽性之病人。回溯其病歷記載、檢驗資料及影像檢查結果分析臨床特徵及血清學表現。結果：總共有45名病人收入此研究。其中7名診斷為多發性肌炎，2位為皮肌炎，2位為無肌炎表現的間質性肺炎。臨床症狀表現上最多的是關節症狀和乾燥症候：在肌炎／間質性肺炎病人群分佔了90.9%及45.5%，在非肌炎／間質性肺炎病人群分佔32.3%及44.1%。雷諾氏現象、發燒和皮膚紅疹表現在肌炎／間質性肺炎病人群有27.2%、45.5%和36.4%；在非肌炎／間質性肺炎病人群則有27.2%、17.6%和17.6%。此45名病人在血清學上總共有22名呈抗核抗體陽性，10名呈風濕因子陽性。在34名非肌炎／間質性肺炎的病人中，臨床表現相當多樣化。結論：本研究發現Jo-1抗體除了典型表現在特發性發炎性肌炎，亦可能有其他多樣性的臨床表現。此外，抗核抗體陰性無法排除Jo-1抗體之存在。

Objective: To elucidate the clinical and serological features of patients with anti-Jo-1 antibodies. Methods: Between 2007 and 2010, patients with positive anti-Jo-1 antibodies were enrolled in a study conducted at National Taiwan University Hospital. Their clinical and serological features were obtained by reviewing medical records, laboratory databases, and image studies. Results: On the basis of the information review, 45 patients with positive anti-Jo-1 antibodies were identified. Seven patients had polymyositis, 2 patients had dermatomyositis, and 2 patients had interstitial lung disease (ILD) without evidence of myositis. The most frequent symptoms were joint manifestation and sicca syndrome: 90.9% patients in the myositis/ILD group and 32.3% patients in the non-myositis/ILD group showed joint manifestation, and 45.5% patients in the myositis/ILD group and 44.1% patients in the non-myositis/ILD group had sicca syndrome. Raynaud’s phenomenon, fever, and skin rash occurred in 27.2%, 45.5%, and 36.4% of the patients in the myositis/ILD group, and in 20.5%, 17.6%, and 17.6% of the patients in the non-myositis/ILD group. Serological profiles of the patients showed positive antinuclear antibody in 22 patients and positive rheumatoid factor in 10 patients. The initial presentations of 34 patients without myositis or ILD were recorded; the presentations were very heterogeneous. Conclusion: Anti-Jo-1 antibody is one of the most important autoantibodies in idiopathic inflammatory myopathies. In our study, patients with anti-Jo-1 antibodies had highly variable clinical presentations. Our findings also revealed that negative or low titers in antinuclear antibody testing could not exclude the presence of anti-Jo-1 antibody.