为了调查青少年特发性炎症性肌病 (IIM) 和成人 IIM 之间自身抗体、临床特征和长期结果的差异，方法：对 320 名受试者的自身抗体、临床特征和最长 20 年的无药物状况进行回顾性分析使用京都大学登记处的日本 IIM 患者（青少年 IIM，n = 34；成人 IIM，n = 286）。在青少年 IIM 中观察到的自身抗体为抗 TIF1-γ (15 %)、抗 MDA-5 (15 %)、抗 ARS (9 %) 和抗 NXP-2 (6 %)。在成人 IIM 中观察到的抗体包括抗 ARS (32%)、抗 MDA-5 (23%)、抗 TIF1-γ (8%)、抗 SRP (8%)、抗 Mi-2 (2 %) 和抗 NXP-2 (1 %)。 20 年内，青少年 IIM 的累积无药物状况率高于成人 IIM（青少年 IIM 与成人 IIM，34% vs. 18%，p = 0.0016）。与成人 IIM 相比，抗 TIF1-γ 与青少年 IIM 中较少的肌肉症状（60% vs. 90%）、恶性肿瘤（0% vs. 57%）和糖皮质激素使用（40% vs. 86%）相关，而青少年 IIM 更多地达到了无药物条件（60% vs. 25%）。具有抗 MDA-5 的青少年 IIM 和成人 IIM 均表现出无肌病性皮肌炎、间质性肺病 (ILD) 和多重免疫抑制治疗的高频率，且无药物情况较高（50 % vs. 49 %）。抗 ARS 治疗的青少年 IIM 和成人 IIM 均表现出频繁的皮疹、肌肉症状和 ILD，频繁需要多种免疫抑制治疗，并且无药物治疗率较低（0 % 与 3 %）。抗NXP-2的青少年-IIM和成人-IIM均表现出频繁的皮疹和肌肉症状、ILD频率较低、频繁使用甲氨蝶呤和糖皮质激素，未达到无药物条件（0 % vs. 0 %）与成人 IIM 患者相比，青少年 IIM 患者更容易达到无药物状态。特定自身抗体与青少年 IIM 和成人 IIM 之间的不同临床特征和结果相关。版权所有 © 2024 Elsevier Inc. 保留所有权利。

To investigate differences in autoantibodies, clinical features, and long-term outcomes between juvenile-idiopathic inflammatory myopathy (IIM) and adult-IIM METHODS: Autoantibodies, clinical characteristics, and drug-free conditions for a maximum of 20 years were retrospectively analyzed in 320 Japanese IIM patients (juvenile-IIM, n = 34; adult-IIM, n = 286) using the Kyoto University Registry.Autoantibodies observed in juvenile-IIM were anti-TIF1-γ (15 %), anti-MDA-5 (15 %), anti-ARS (9 %), and anti-NXP-2 (6 %). Those observed in adult-IIM were anti-ARS (32 %), anti-MDA-5 (23 %), anti-TIF1-γ (8 %), anti-SRP (8 %), anti-Mi-2 (2 %), and anti-NXP-2 (1 %). The cumulative drug-free condition rate was higher in juvenile-IIM than in adult-IIM up to 20 years (juvenile-IIM vs. adult-IIM, 34 % vs. 18 %, p = 0.0016). Anti-TIF1-γ was associated with lesser muscle symptoms (60 % vs. 90 %), malignancy (0 % vs. 57 %), and glucocorticoid use (40 % vs. 86 %) in juvenile-IIM compared to adult-IIM, while juvenile-IIM more achieved drug-free conditions (60 % vs. 25 %). Both juvenile-IIM and adult-IIM with anti-MDA-5 demonstrated a high frequency of amyopathic dermatomyositis, interstitial lung disease (ILD), and multi-immunosuppressive therapy, with high drug-free conditions (50 % vs. 49 %). Both juvenile-IIM and adult-IIM with anti-ARS showed frequent skin rashes, muscle symptoms, and ILD, frequent need for multi-immunosuppressive therapy, and low drug-free condition rates (0 % vs. 3 %). Both juvenile-IIM and adult-IIM with anti-NXP-2 showed frequent skin rashes and muscle symptoms, low ILD frequency, and frequent use of methotrexate and glucocorticoids, which did not achieve drug-free conditions (0 % vs. 0 %).Drug-free condition was achieved more frequently in juvenile-IIM patients than adult-IIM patients. Specific autoantibodies were associated with different clinical characteristics and outcomes between juvenile-IIM and adult-IIM.Copyright © 2024 Elsevier Inc. All rights reserved.