Department of Immunology, Instituto de Investigaciones Médicas Alfredo Lanari, Buenos Aires University, Autonomous City of Buenos Aires, 3150 Combatientes de Malvinas Ave, 1431, PC, Argentina.
Department of Rheumatology, Hospital de Clínicas, José de San Martín, Buenos Aires University, Autonomous City of Buenos Aires, Argentina.
Clin Rheumatol. 2021 Nov;40(11):4473-4483. doi: 10.1007/s10067-021-05797-2. Epub 2021 Jun 22.
INTRODUCTION/OBJECTIVES: To describe clinical features in patients with inflammatory myopathies (IMs) from the Argentine Registry of Inflammatory Myopathies, and their relationship with myositis-specific antibodies (MSAs).
This cross-sectional study included 360 adult patients with dermatomyositis (DM), polymyositis (PM), and inclusion body myositis. Demographics, clinical, and serological characteristics were retrospectively recorded (2016-2019). MSAs were determined by immunoblotting. Patients who were positive for anti-Jo-1, Mi-2, and MDA5 were compared against a group of patients, taken as reference group, who were negative for all MSAs.
Women 72%, median age at diagnosis was 47.3 years (18-82). The most frequent subtypes were DM (43.9%) followed by PM (30%).The most frequent MSAs were anti-Jo-1 (51/317), 16.1%; MDA5 (12/111), 10.8%, and Mi-2 (23/226), 10.2%. Anti-Jo-1 was associated (p < 0.05) with a higher frequency of chronic disease course, interstitial lung disease (ILD), arthritis, and mechanic's hands. Anti-Mi-2 was found in patients who had higher frequency of skin manifestations and higher CK values (p < 0.001). Patients with anti-MDA5 had normal or low CK levels. Anti-MDA5 was associated (p < 0.05) with skin manifestations, arthritis, and ILD. The rest of MSAs had frequencies lower than 8%. Anti-TIF1ϒ was found in eight DM patients and one had cancer. Anti-SRP was found in seven patients who had PM and elevated CK.
Anti-Jo-1 was the most frequent MSA, and was associated with ILD; MDA5 was associated with CADM and ILD, and Mi-2, with classical DM. Despite the different prevalence with respect to other cohorts, the clinical characteristics for each MSA group were similar to the data reported in other studies. Key Points • This study describes the prevalence of MSAs in the Argentine Registry of IMs. • Anti-Jo-1 and anti-MDA5 were associated with ILD. • Anti-Mi-2 was the third most frequent MSA, associated with classical DM.
介绍/目的：描述来自阿根廷炎症性肌病登记处的炎症性肌病（IM）患者的临床特征及其与肌炎特异性抗体（MSA）的关系。
这项横断面研究纳入了 360 名成年皮肌炎（DM）、多发性肌炎（PM）和包涵体肌炎患者。回顾性记录人口统计学、临床和血清学特征（2016-2019 年）。通过免疫印迹法确定 MSA。与一组所有 MSA 均为阴性的患者（作为参考组）相比，抗 Jo-1、Mi-2 和 MDA5 阳性的患者被进行比较。
女性占 72%，中位发病年龄为 47.3 岁（18-82 岁）。最常见的亚型是 DM（43.9%），其次是 PM（30%）。最常见的 MSA 是抗 Jo-1（51/317），占 51/317，16.1%；MDA5（12/111），占 10.8%；Mi-2（23/226），占 10.2%。抗 Jo-1 与慢性病程（p<0.05）、间质性肺病（ILD）、关节炎和技工手的发生率较高相关。抗 Mi-2 存在于皮肤表现和 CK 值较高的患者中（p<0.001）。抗 MDA5 患者的 CK 值正常或较低。抗 MDA5 与皮肤表现、关节炎和 ILD 相关（p<0.05）。其余 MSA 的频率低于 8%。8 例 DM 患者和 1 例癌症患者中发现抗 TIF1γ。7 例 PM 患者和 CK 升高患者中发现抗 SRP。
抗 Jo-1 是最常见的 MSA，与 ILD 相关；MDA5 与 CADM 和 ILD 相关，Mi-2 与经典 DM 相关。尽管与其他队列的流行率不同，但每个 MSA 组的临床特征与其他研究报告的数据相似。
关键点
·本研究描述了阿根廷炎症性肌病登记处的 MSA 患病率。
·抗 Jo-1 和抗 MDA5 与 ILD 相关。
·抗 Mi-2 是第三种最常见的 MSA，与经典 DM 相关。
