Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy
Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy
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DOI：
10.1002/art.11484
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发表时间：
2004-01-01
影响因子：
--
通讯作者：
Oddis, CV
中科院分区：
文献类型：
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作者：
Kao, AH;Lacomis, D;Oddis, CV
Objective.确定结缔组织病（CTD）和抗信号识别颗粒（抗SRP）自身抗体患者队列的长期结局和相关临床、血清学和病理学特征。前瞻性地从患有多发性肌炎（PM; n = 134）、皮肌炎（n = 129）或其他CTD（主要是系统性硬化症[SSc; n = 790]）的连续成人患者中收集血清和临床数据。1973年至2001年首次对患者进行评估。19例抗SRP自身抗体患者被确定，其中16例（84%）为纯PM，3例（2例SSc和1例抗合成酶综合征）在平均随访4.5年（范围2.5-6年）后尚未出现肌炎特征。与抗合成酶阳性PM对照组相比，更多SRP阳性PM患者在初始表现时有严重的近端肌无力（50%）和肌肉萎缩（67%）。16例SRP阳性PM患者中仅2例（13%）发生心脏受累，13例SRP阳性PM患者中有3例（23%）和3例SRP阳性非肌炎患者发生间质性肺病。SRP阳性PM队列的肌肉活检标本中相对缺乏炎症。其他自身抗体包括Ro/SSA（4例）、Th/To（1例）和抗PL-12（1例）。SRP阳性PM患者的生存率与SRP阴性PM患者的生存率相当。抗SRP自身抗体对PM无特异性。严重的肌无力和萎缩是抗SRP的PM患者的突出特征。心脏受累较不常见，抗SRP患者的生存率比以前报道的更好。
Objective. To determine the long-term outcome and associated clinical, serologic, and pathologic features in a cohort of patients with connective tissue disease (CTD) and the anti-signal recognition particle (anti-SRP) autoantibody.Methods. Sera and clinical data were collected prospectively from consecutive adult patients with polymyositis (PM; n = 134), dermatomyositis (n = 129), or other CTDs (predominantly systemic sclerosis [SSc; n = 790]). Patients were first evaluated during 1973-2001.Results. Nineteen patients with the anti-SRP autoantibody were identified, 16 (84%) of whom had pure PM and 3 (2 with SSc and 1 with antisynthetase syndrome) had yet to develop features of myositis after a mean followup of 4.5 years (range 2.5-6 years). More SRP-positive PM patients had severe proximal muscle weakness (50%) and muscle atrophy (67%) at initial presentation compared with antisynthetase-positive PM controls. Cardiac involvement occurred in only 2 of 16 SRP-positive PM patients (13%), and interstitial lung disease was noted in 3 of 13 SRP-positive PM patients (23%) and in the 3 SRP-positive nonmyositis patients. There was a relative lack of inflammation in muscle biopsy specimens from the SRP-positive PM cohort. Other autoantibodies in the SRP-positive patients included Ro/SSA (4 patients), Th/To (I patient), and anti-PL-12 (1 patient). Survival in the SRP-positive PM patients was comparable with that seen in the cohort of SRP-negative PM patients.Conclusion. The anti-SRP autoantibody is not specific for PM. Severe muscle weakness and atrophy were prominent features in PM patients with anti-SRP. Cardiac involvement was less common and survival was better in patients with anti-SRP than has previously been reported.