Peking University, China-Japan Friendship School of clinical medicine, Beijing, China.
Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.
Front Immunol. 2022 Aug 30;13:987841. doi: 10.3389/fimmu.2022.987841. eCollection 2022.
To explore the clinical features and prognoses of dermatomyositis (DM) associated with a double-positive anti-MDA5 and anti-aminoacyl-tRNA synthetase (anti-ARS) antibody presentation.
We retrospectively analyzed 1280 consecutive patients with idiopathic inflammatory myopathy (IIM). Individuals with anti-MDA5 and anti-ARS antibodies (anti-MDA5+/ARS+) were compared to anti-MDA5-/ARS+ and anti-MDA5+/ARS- control individuals based on clinical, pulmonary radiological characteristics, treatment, and follow-up information.
Six individuals (0.47%) presented with anti-MDA5+/ARS+; of these, 2 (33.3%) were anti-PL-12+, 2 (33.3%) were anti-Jo-1+, 1 (16.7%) was anti-EJ+, and 1 (16.7%) was anti-PL-7+. Hallmark cutaneous manifestations, including Gottron's sign (100%), heliotrope rash (50%), mechanic's hand (66.7%), and skin ulcers (16.7%) were common. Anti-MDA5+/ARS+ patients tended to have higher ferritin levels (p = 0.038) than anti-MDA5-/ARS+ group, and higher CD4+ T-cell counts (p = 0.032) compared to the anti-MDA5+/ARS- group. Radiologically, NSIP with OP overlap was predominant (60%). Consolidation (60%), ground-glass attenuation (GGA) (80%), traction bronchiectasis (80%), and intralobular reticulation (100%) were common in anti-MDA5+/ARS+ individuals. All were diagnosed with ILD and 50% were categorized as RPILD. All patients received glucocorticoids combined with one or more immunosuppressants. Most (83.3%) had a good prognosis following treatment, but there was no difference in the survival rate between the three subgroups.
Presentation with anti-MDA5+/ARS+ DM was rare. The clinical and radiological characteristics of anti-MDA5+/ARS+ DM combined the features of anti-MDA5+ and anti-ARS+ individuals. Individuals with anti-MDA5+/ARS+ antibodies may respond well to glucocorticoid therapy; glucocorticoids combined with one or more immunosuppressants may be considered a basic treatment approach.
探讨抗 MDA5 和抗氨酰-tRNA 合成酶（抗-ARS）抗体双重阳性的皮肌炎（DM）的临床特征和预后。
我们回顾性分析了 1280 例特发性炎性肌病（IIM）连续患者。根据临床、肺部影像学特征、治疗和随访信息，将抗 MDA5 和抗 ARS 抗体阳性个体（抗 MDA5+/ARS+）与抗 MDA5-/ARS+和抗 MDA5+/ARS-对照组个体进行比较。
6 例（0.47%）出现抗 MDA5+/ARS+；其中，2 例（33.3%）为抗 PL-12+，2 例（33.3%）为抗 Jo-1+，1 例（16.7%）为抗 EJ+，1 例（16.7%）为抗 PL-7+。常见的特征性皮肤表现包括 Gottron 征（100%）、向阳疹（50%）、技工手（66.7%）和皮肤溃疡（16.7%）。抗 MDA5+/ARS+患者的铁蛋白水平（p=0.038）高于抗 MDA5-/ARS+组，CD4+T 细胞计数（p=0.032）高于抗 MDA5+/ARS-组。影像学上，NSIP 与 OP 重叠为主（60%）。实变（60%）、磨玻璃影（GGA）（80%）、牵引性支气管扩张（80%）和小叶内网状影（100%）在抗 MDA5+/ARS+患者中常见。所有患者均诊断为ILD，50%为 RPILD。所有患者均接受糖皮质激素联合一种或多种免疫抑制剂治疗。治疗后大多数（83.3%）预后良好，但三组患者的生存率无差异。
抗 MDA5+/ARS+DM 表现罕见。抗 MDA5+/ARS+DM 的临床和影像学特征结合了抗 MDA5+和抗 ARS+个体的特征。抗 MDA5+/ARS+抗体患者对糖皮质激素治疗反应良好；可考虑糖皮质激素联合一种或多种免疫抑制剂作为基本治疗方法。
