Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy
Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy
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DOI：
10.1136/ard.2006.052191
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发表时间：
2006-12-01
影响因子：
27.4
通讯作者：
van Engelen, B. G. M.
中科院分区：
文献类型：
--
作者：
Hengstman, G. J. D.;ter Laak, H. J.;van Engelen, B. G. M.
目的：探讨抗信号识别颗粒（SRP）自身抗体在肌炎患者中的临床意义。方法：对来自欧洲6个中心的23例抗srp阳性患者的临床、实验室和组织学特征进行回顾性系统评估。数据与先前发表的一大组抗srp阴性肌炎患者进行比较。结果：临床中，抗srp自身抗体患者常出现严重的对称性近端肌无力，导致明显的残疾、吞咽困难和血清肌酸激酶水平高。3例患者出现典型皮肌炎皮疹。该疾病与肌外体征和症状的发生有关，包括间质性肺疾病。没有发现与心脏受累风险增加相关，并且大多数患者对治疗有反应，该疾病具有合理的良好预后。所有患者均无肌炎的典型组织学特征。大多数肌肉活检标本显示存在坏死肌纤维，无炎症浸润。结论：抗srp自身抗体与免疫介导的肌病谱系中的坏死性肌病综合征相关，不同于典型的多发性肌炎。需要进一步的研究来阐明其发病机制，并阐明抗srp自身抗体在这种独特疾病中的作用。
Objective: To elucidate the clinical importance of the anti-signal recognition particle (SRP) autoantibody in patients with myositis.Methods: Retrospective systematic assessment of the clinical, laboratory and histological characteristics of 23 anti-SRP-positive patients from six European centres. Data were compared with a large group of anti-SRP-negative patients with myositis published previously.Results: Clinically, patients with anti-SRP autoantibodies often had a severe symmetric proximal muscle weakness resulting in marked disability, dysphagia and highly elevated levels of serum creatine kinase. Three patients had typical dermatomyositis rashes. The disease was associated with the occurrence of extramuscular signs and symptoms including interstitial lung disease. No association was found with an increased risk of cardiac involvement, and the disease carried a reasonably favourable prognosis with most patients responding to treatment. None of the patients had the typical histological features of myositis. Most muscle biopsy specimens showed the presence of necrotic muscle fibres and no inflammatory infiltrates.Conclusions: Anti-SRP autoantibodies are associated with a syndrome of a necrotising myopathy in the spectrum of immune-mediated myopathies that differs from typical polymyositis. Further studies are needed to elucidate the pathogenesis and to clarify the role of the anti-SRP autoantibodies in this unique disease.