Kagawa Hiroyuki, Tsujino Kazuyuki, Yamamoto Yuji, Iwai Ami, Hara Reina, Matsuki Takanori, Fukushima Kiyoharu, Oshitani Yohei, Yoshimura Kenji, Miki Mari, Miki Keisuke, Kitada Seigo, Mori Masahide, Kida Hiroshi
Department of Respiratory Medicine, National Hospital Organization Osaka Toneyama Medical Center, 5-1-1 Toneyama, Toyonaka, Osaka, 560-8552, Japan.
Respir Med Case Rep. 2020 Feb 1;29:101016. doi: 10.1016/j.rmcr.2020.101016. eCollection 2020.
The presence of anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is closely associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis. Despite intensive immunosuppressive therapies, some of these patients still have a poor prognosis with few treatment options. Although removal of pathogenic autoantibodies and cytokines by plasma exchange (PE) could be a treatment option, its safety and efficacy have never been determined. We report a patient with anti-MDA5 Ab-positive RP-ILD who was refractory to intensive therapies including steroids, cyclosporine, and intravenous cyclophosphamide, and then treated by PE to prevent the progression of RP-ILD. Shortly after the initiation of PE therapy, however, his respiratory condition suddenly deteriorated due to acute pulmonary edema and the patient died on the following day. Transfusion-related acute lung injury (TRALI) would be the most likely cause of the acute pulmonary edema because there was no sign of circulatory overload. To the best of our knowledge, this is the first report showing a critical adverse event associated with PE therapy for these patients. This case supports the idea that the presence of ILD could increase a risk for TRALI and therefore we should carefully evaluate the eligibility for PE therapy of anti-MDA5 Ab-positive RP-ILD patients given the risk of acute lung injury. Further studies collecting more clinical data are necessary to assess the efficacy, safety, and risk factors of PE therapy for these patients.
抗黑色素瘤分化相关基因5抗体（抗MDA5抗体）的存在与临床无肌病性皮肌炎患者的快速进展性间质性肺病（RP-ILD）密切相关。尽管进行了强化免疫抑制治疗，但这些患者中的一些人预后仍然很差，治疗选择很少。虽然通过血浆置换（PE）去除致病性自身抗体和细胞因子可能是一种治疗选择，但其安全性和有效性尚未得到确定。我们报告了一名抗MDA5抗体阳性的RP-ILD患者，他对包括类固醇、环孢素和静脉注射环磷酰胺在内的强化治疗无效，随后接受PE治疗以防止RP-ILD进展。然而，在PE治疗开始后不久，他的呼吸状况因急性肺水肿突然恶化，患者于次日死亡。输血相关急性肺损伤（TRALI）很可能是急性肺水肿的原因，因为没有循环超负荷的迹象。据我们所知，这是第一份显示与这些患者的PE治疗相关的严重不良事件的报告。该病例支持ILD的存在可能增加TRALI风险的观点，因此鉴于急性肺损伤的风险，我们应该仔细评估抗MDA5抗体阳性RP-ILD患者PE治疗的适用性。需要进一步收集更多临床数据的研究来评估这些患者PE治疗的疗效、安全性和危险因素。
