Division of Rheumatology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand.
Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Clin Rheumatol. 2019 Dec;38(12):3459-3469. doi: 10.1007/s10067-019-04756-2. Epub 2019 Aug 24.
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.
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.
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).
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.Key Points• NAM is indistinguishable from non-NAM by clinical manifestations, serology, or laboratory findings.• The integration of clinical, serological, and pathological data is essential for making a diagnosis of NAM.
研究泰国患者与其他特发性炎性肌病（IIM）或非坏死性自身免疫性肌病（NAM）患者之间的临床特征、实验室特征和治疗结果的差异。
本多中心病例对照研究纳入了年龄≥18 岁、经肌肉病理诊断为 IIM 且至少有 3 次随访的患者，随访时间为 1 年。记录基线临床和实验室数据。招募当天采集血清肌炎特异性自身抗体（MSA）。
70 例患者中，67%为 NAM，33%为非 NAM。患者平均年龄为 50.5±15.9 岁，67%为女性，中位症状持续时间为 2 个月（IQR，1-4）。非 NAM 患者癌症史发生率明显较高（21.7%比 2.1%，p=0.01）。非 NAM 患者 Gottron 丘疹更为常见（21.7%比 4.3%，p=0.04）。非 NAM 患者抗 Mi-2a（17.4%比 2.1%，p=0.04）和 Mi-2b（17.4%比 0.0%，p=0.01）的阳性率更高；然而，两组间其他 MSA，包括抗 HMGCR 和抗 SRP，的存在情况相似。在整个随访期间，3 次强化糖皮质激素和/或免疫抑制剂治疗，改善运动功能的患者比例在两组间相似（NAM 46.8%比非 NAM 34.8%，p=0.34）。
NAM 在临床表现、血清学或实验室检查方面与非 NAM 无法区分，除了具有特征性的 Gottron 丘疹皮肤表现和抗 Mi2 有助于诊断皮肌炎。整合临床、血清学和病理学数据对于诊断 NAM 至关重要。
NAM 在临床表现、血清学或实验室检查方面与非 NAM 无法区分。
整合临床、血清学和病理学数据对于诊断 NAM 至关重要。
