Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age
Clinical features of dermatomyositis associated with anti-MDA5 antibodies by age
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DOI：
10.1080/14397595.2020.1740400
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发表时间：
2020-03-28
影响因子：
2.2
通讯作者：
Maeno, Toshitaka
中科院分区：
文献类型：
--
作者：
Yamaguchi, Koichi;Yamaguchi, Aya;Maeno, Toshitaka
目的：抗黑色素瘤分化相关基因5（MDA 5）自身抗体阳性和发病年龄≥ 60岁是日本患者中与间质性肺病（ILD）相关的多发性肌炎（PM）和皮肌炎（DM）的不良预后因素。然而，年龄对抗MDA 5自身抗体阳性的DM患者的临床特征的影响仍不清楚。方法：回顾性分析40例DM患者抗MDA 5自身抗体的临床资料。我们比较了年龄≥ 60岁患者的临床特征，包括实验室检查结果、高分辨率肺部计算机断层扫描数据、治疗内容和并发症（如感染和预后）。我们还研究了老年患者组中存活和死亡患者之间的临床特征。结果：40例患者中，13例为老年人，27例为年轻人。老年患者的临床症状包括关节痛/关节炎（p <0.01）、皮肤溃疡（p = 0.02）明显少于年轻患者（p = 0.02），并发快速进展性ILD（RP-ILD）、联合免疫抑制治疗和严格控制的感染的死亡率更高。结论：年龄影响抗-MDA 5自身抗体阳性DM患者的临床特征和死亡率。年龄≥ 60岁的患者预后较差，联合免疫抑制治疗对老年患者的RP-ILD通常无效。
Objectives: Anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody-positive and age at onset >= 60 years are poor prognosis factors in polymyositis (PM) and dermatomyositis (DM) associated with interstitial lung disease (ILD) among Japanese patients. However, the influence of age on the clinical features of anti-MDA5 autoantibody-positive patients with DM remains unclear. Methods: We retrospectively examined 40 patients with DM and anti-MDA5 autoantibodies according to age. We compared patients aged = 60 years with respect to clinical features including laboratory test findings, high-resolution lung computed tomography data, treatment content, and complications such as infections and prognosis. We also examined clinical features between surviving and deceased patients in the older patient group. Results: Of 40 enrolled patients, 13 were classified as old and 27 as young. Older patients had significantly fewer clinical symptoms including arthralgia/arthritis (p < .01), skin ulceration (p = .02), and higher mortality than younger patients (p = .02) complicated with rapidly progressive ILD (RP-ILD), combination immunosuppressive therapy, and strictly controlled infections. Conclusion: Clinical features and mortality of anti-MDA5 autoantibody-positive DM patients were influenced by age. Patients aged >= 60 years had a worse prognosis, and combination immunosuppressive therapy was often ineffective for RP-ILD in older patients.