【摘要】 目的测定多发性肌炎(PM)/皮肌炎(DM)患者血清中肌炎特异性抗体(MSA)及肌炎相关性抗体(MAA)的阳性率,探讨MSA在PM/DM中的意义。方法PM/DM患者31例,用免疫印迹法检测血清MSAs和MAAs阳性率,分析患者的临床表现及预后与自身抗体的相关性。结果31例患者中18例出现MSAs和/或MAAs阳性(58%),其中MSAs阳性率为39%,最常见的MSAs是抗Jo-1抗体(29%),其次是抗Mi-2抗体(6%),抗PL-7抗体(3%),抗PL-12抗体(3%),MAAs的阳性率为32%,其中抗Ku-72占16%,抗Ku-86占23%,抗PM-Scl抗体占6%。肺间质病变及关节炎/痛在抗Jo-1抗体阳性患者中的比例明显高于抗Jo-1抗体阴性组(P<0.05)。治疗后18例患者完全缓解,12例部分缓解,1例死亡。随访12例患者,在激素减量后均有复发,MSAs阳性与阴性组间复发次数、病死率无明显差别。肺间质纤维化在MSAs阳性患者中发生率较高,预后较差。结论MSAs和MAAs在PM/DM患者中阳性表达率较高,MSAs与PM/DM的临床表现和预后相关。开展MSAs及MAAs的测定对于PM/DM临床诊断和治疗有重要意义。更多还原

【Abstract】 Objective To detect the prevalence of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in patients with polymyositis (PM) and dermatomyositis (DM), and analyse the correlation between MSAs and the clinical features and prognosis of PM/DM. Methods Serum samples of 31 PM and DM patients were screened for MSAs (including anti Jo-1, anti Mi-2, anti PL-7, anti PL-12 antibodies) and MAAs (including anti Ku, anti PM-Scl antibodies) by immunoblotting test. Results Serum MSAs/MAAs were detected in 18 out of 31 PM/DM patients (58%). MSAs were present in 12 patients (39%). The most frequently encountered MSAs was anti-Jo-1 autoantibody (29%), followed by anti-Mi-2 (7%), anti-PL-7 (3%), and anti-PL-12 (3%). MAAs were present in 10 patients (32%), including anti-Ku-72 (16%), anti-Ku-86 (23%) and anti-PM/Scl (7%). Notably, anti-Jo-1 antibody was closely associated with interstitial lung disease (ILD) and arthritis/arthralgia compared with the anti-Jo-1 antibody negative patients (P<0.05). Treatment of corticosteroid plus immunosuppressive drugs was effective in achieving complete remission in 18 patients and partial remission in 12 patients, but relapse was frequent during follow-up (1~12 years). The relapse frequency and mortality were similar between MSAs positive and negative groups. The incidence of ILD was significantly increased in MSAs positive patients with a poor prognosis. Conclusions Our data indicate that the prevalence of MSAs and MAAs are high in PM/DM patients. MSAs are associated with clinical features and prognosis in patients with PM and DM. The determination of MSAs/MAAs has important implication for clinical diagnosis and treatment of PM and DM.更多还原