Abstract：
OBJECTIVE: 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.
RESULTS: 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 %).
CONCLUSIONS: 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.
RESULTS: 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 %).
CONCLUSIONS: 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.
摘要：
目的：为了研究自身抗体的差异，临床特征,青少年特发性炎性肌病（IIM）和成人IIM之间的长期结果方法：自身抗体，临床特征,对320名日本IIM患者（青少年IIM，n=34；成人IIM，n=286)使用京都大学登记处。
结果：在青少年IIM中观察到的自身抗体是抗TIF1-γ（15％），抗MDA-5（15%），反ARS（9%），和反NXP-2（6％）。在成人IIM中观察到的是抗ARS（32％），抗MDA-5（23%），抗TIF1-γ（8％），反SRP（8%），反Mi-2（2%），和反恩智浦-2（1％）。青少年IIM的累积无药状态率高于成人IIM，直至20年（青少年IIM与成人-IIM,34%vs.18%,p=0.0016)。抗TIF1-γ与较轻的肌肉症状相关(60%vs.90%)，恶性肿瘤(0%与57%），和糖皮质激素的使用（40％与86％）与成人IIM相比，青少年IIM，而青少年-IIM更多地实现了无毒品条件(60%vs.25%）。使用抗MDA-5的青少年IIM和成人IIM均表现出高频率的肌病性皮肌炎，间质性肺病（ILD），和多种免疫抑制疗法，高无毒条件(50%vs.49%)。患有抗ARS的青少年IIM和成人IIM均表现出频繁的皮疹，肌肉症状,ILD,经常需要多种免疫抑制治疗，和低无药率(0%vs.3%)。使用抗NXP-2的青少年IIM和成人IIM都表现出频繁的皮疹和肌肉症状，低ILD频率，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%)。
结论：与成年IIM患者相比，青少年IIM患者获得无药状态的频率更高。特异性自身抗体与青少年IIM和成人IIM之间不同的临床特征和结果相关。
结果：在青少年IIM中观察到的自身抗体是抗TIF1-γ（15％），抗MDA-5（15%），反ARS（9%），和反NXP-2（6％）。在成人IIM中观察到的是抗ARS（32％），抗MDA-5（23%），抗TIF1-γ（8％），反SRP（8%），反Mi-2（2%），和反恩智浦-2（1％）。青少年IIM的累积无药状态率高于成人IIM，直至20年（青少年IIM与成人-IIM,34%vs.18%,p=0.0016)。抗TIF1-γ与较轻的肌肉症状相关(60%vs.90%)，恶性肿瘤(0%与57%），和糖皮质激素的使用（40％与86％）与成人IIM相比，青少年IIM，而青少年-IIM更多地实现了无毒品条件(60%vs.25%）。使用抗MDA-5的青少年IIM和成人IIM均表现出高频率的肌病性皮肌炎，间质性肺病（ILD），和多种免疫抑制疗法，高无毒条件(50%vs.49%)。患有抗ARS的青少年IIM和成人IIM均表现出频繁的皮疹，肌肉症状,ILD,经常需要多种免疫抑制治疗，和低无药率(0%vs.3%)。使用抗NXP-2的青少年IIM和成人IIM都表现出频繁的皮疹和肌肉症状，低ILD频率，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%)。
结论：与成年IIM患者相比，青少年IIM患者获得无药状态的频率更高。特异性自身抗体与青少年IIM和成人IIM之间不同的临床特征和结果相关。
