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首页> 外文期刊>Clinical rheumatology >Clinical spectrums and outcomes of necrotizing autoimmune myopathy versus other idiopathic inflammatory myopathies: a multicenter case-control study
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Clinical spectrums and outcomes of necrotizing autoimmune myopathy versus other idiopathic inflammatory myopathies: a multicenter case-control study

机译：坏死性自身免疫肌病的临床谱和结果与其他特发性炎症性肌病：多中心病例对照研究

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Objective To investigate the clinical characteristics, laboratory features, and treatment outcomes of Thai patients compared between those with necrotizing autoimmune myopathy (NAM) and those with other idiopathic inflammatory myopathies (IIMs) or non-NAM. Methods This multicenter case-control study included patients aged >= 18 years who were diagnosed with IIMs by muscle pathology, and who had relevant clinical and laboratory data, including muscle enzymes, from at least 3 follow-up visits during a 1-year period. Baseline clinical and laboratory data were recorded. Serum myositis-specific autoantibodies (MSAs) were obtained on the date of recruitment. Results Of the 70 included patients, 67% had NAM, and 33% had non-NAM. The mean age of patients was 50.5 +/- 15.9 years, 67% were female, and the median duration of symptoms was 2 months (IQR, 1-4). History of cancer was significantly higher in non-NAM (21.7% vs. 2.1%, p = 0.01). Gottron's papules were significantly more prevalent in non-NAM (21.7% vs. 4.3%, p = 0.04). Non-NAM had a higher prevalence of anti-Mi-2a (17.4% vs. 2.1%, p = 0.04) and Mi-2b (17.4% vs. 0.0%, p = 0.01); however, the presence of other MSAs, including anti-HMGCR and anti-SRP, was similar between groups. Improvement in motor power and treatment intensification with glucocorticoid and/or immunosuppressive agents 3 times throughout the follow-up period was similar between groups (NAM 46.8% vs. non-NAM 34.8%, p = 0.34). Conclusion NAM is indistinguishable from non-NAM by clinical manifestations, serology, or laboratory findings, except that pathognomonic skin sign of Gottron's papules and anti-Mi2 are suggestive of dermatomyositis. The integration of clinical, serological, and pathological data is essential for making a diagnosis of NAM.
机译：目的探讨泰国患者的临床特征，实验室特征和治疗结果，与坏死性自身免疫疗病（NAM）和其他特发性炎症肌病（IIMS）或非NAM的患者之间的临床特征，实验室特征和治疗结果。方法该多中心病例对照研究包括肌肉病理学诊断患者的患者> = 18岁，并在1年期间，有相关的临床和实验室数据，包括肌肉酶，包括肌肉酶，从1年期间至少进行3次。记录基线临床和实验室数据。在招聘日期获得血清肌炎特异性自身抗体（MSAS）。 70款患者的结果67％有NAM，33％的非NAM。患者的平均年龄为50.5 +/- 15.9岁，67％是女性，症状的中位持续时间为2个月（IQR，1-4）。癌症的历史在非Nam（21.7％vs.2.1％，P = 0.01）显着高。 Gottron的丘疹在非NAM中具有显着普遍的（21.7％与4.3％，P = 0.04）。非NAM具有更高的抗MI-2A（17.4％，P = 0.04）和MI-2B的患病率较高（17.4％，对0.0％，P = 0.01）;然而，在组之间存在其他MSA的存在，包括抗HMGCR和抗SRP。在整个随访期间，在整个后续期间的3次与糖皮质激素和/或免疫抑制剂的改善在整个随访期间相似（Nam 46.8％与非Nam 34.8％，P = 0.34）。结论NAM临床表现，血清学或实验室发现与非NAM难以区分，不同之处在于Gottron的丘疹的病例皮肤迹象和抗MI2暗示皮肤肌炎。临床，血清学和病理数据的整合对于诊断NAM至关重要。

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