Takahashi Rina, Yoshida Tadashi, Morimoto Kohkichi, Kondo Yasushi, Kikuchi Jun, Saito Shuntaro, Ishigaki Sho, Kaneko Yuko, Takeuchi Tsutomu, Itoh Hiroshi, Oya Mototsugu
Division of Endocrinology, Metabolism and Nephrology, Department of Internal Medicine, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.
Apheresis and Dialysis Center, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.
Clin Med Insights Case Rep. 2021 Jul 27;14:11795476211036322. doi: 10.1177/11795476211036322. eCollection 2021.
Patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) are frequently accompanied by rapidly progressive-interstitial lung disease (RP-ILD). They are often refractory to intensive immunosuppressive therapy and have poor prognosis.
A 73-year-old woman presented with fever, cold symptoms, and skin eruption for a month. She also exhibited muscle weakness on upper extremities slightly. The titer of anti-MDA5 antibody was extremely high, and computed tomography showed ground glass opacity and reticular shadows in the lungs. She was diagnosed as anti-MDA5 antibody-positive classical DM-associated RP-ILD and treated with intensive immunosuppressive therapy. However, the titer of anti-MDA5 antibody did not decrease satisfactorily, and plasma exchange was alternatively initiated. The titer decreased remarkably, and she obtained disease remission. Similarly, a 63-year-old woman presented with stiffness of the neck and hands, fever and cough. She was also diagnosed as anti-MDA5 antibody-positive classical DM-associated RP-ILD, because she had skin eruptions, slight muscle weakness, an elevation in anti-MDA5 antibody, and RP-ILD. She was unresponsive to intensive immunosuppressive therapy, but plasma exchange successfully improved the titer of anti-MDA5 antibody, the symptoms, and the findings of computed tomography.
Although anti-MDA5 antibody-positive DM-associated RP-ILD has a high mortality rate, this report suggests the usefulness of plasma exchange to improve the prognosis.
抗黑色素瘤分化相关基因5（MDA5）抗体阳性的皮肌炎（DM）患者常伴有快速进展性间质性肺病（RP-ILD）。他们通常对强化免疫抑制治疗耐药，预后较差。
一名73岁女性，出现发热、感冒症状及皮疹1个月。她还略有上肢肌无力。抗MDA5抗体滴度极高，计算机断层扫描显示肺部有磨玻璃影和网状阴影。她被诊断为抗MDA5抗体阳性的经典DM相关RP-ILD，并接受强化免疫抑制治疗。然而，抗MDA5抗体滴度下降不明显，遂改为进行血浆置换。滴度显著下降，她病情缓解。同样，一名63岁女性，出现颈部和手部僵硬、发热及咳嗽。她也被诊断为抗MDA5抗体阳性的经典DM相关RP-ILD，因为她有皮疹、轻度肌无力、抗MDA5抗体升高及RP-ILD。她对强化免疫抑制治疗无反应，但血浆置换成功改善了抗MDA5抗体滴度、症状及计算机断层扫描结果。
尽管抗MDA5抗体阳性的DM相关RP-ILD死亡率很高，但本报告提示血浆置换对改善预后有用。
