A case of clinically amyopathic dermatomyositis that was refractory to intensive immunosuppressive therapy including tofacitinib, but successfully treated with plasma exchange therapy
A case of clinically amyopathic dermatomyositis that was refractory to intensive immunosuppressive therapy including tofacitinib, but successfully treated with plasma exchange therapy
复制标题
一例临床无肌病性皮肌炎，对包括托法替布在内的强化免疫抑制治疗无效，但通过血浆置换疗法成功治愈
DOI：
10.1093/mrcr/rxab054
复制
发表时间：
2022
期刊：
影响因子：
--
通讯作者：
Hasegawa H.
中科院分区：
文献类型：
--
作者：
Hiraoka D;Ishizaki J;Horie K;Matsumoto T;Suemori K;Takenaka K;Hasegawa H.
临床上无肌病性皮肌炎（CADM）患者经常发展为快速进行性间质性肺病（RP-ILD）。治疗前高水平的抗黑色素瘤分化相关基因5抗体（抗MDA 5 Ab）与RP-ILD的发生、治疗应答差和生存期差相关。CADM患者的预后仍然很差，即使联合强化免疫抑制治疗，由于ILD。最近，几种其他疗法，包括托法替尼（TOF）和血浆置换（PE）治疗，已被报道是有效的。我们在此报告一例CADM-ILD伴高水平抗MDA 5抗体，对包括TOF在内的联合强化免疫抑制治疗无效，但经PE治疗成功。以下是TOF无效的可能原因：（1）未被TOF抑制的细胞因子在RP-ILD中起重要作用;（2）TOF的给药时间晚于先前报道的时间;（3）TOF未抑制抗体等病理物质。另一方面，PE去除细胞因子和各种病理物质。因此，PE可能是难治性CADM-ILD更合理的附加治疗。
Clinically amyopathic dermatomyositis (CADM) patients often develop rapidly progressive interstitial lung disease (RP-ILD). A high level of anti-melanoma differentiation-associated gene 5 antibodies (anti-MDA5 Ab) before treatment is associated with RP-ILD development, a poor treatment response, and poor survival. The prognosis of CADM patients remains poor due to ILD even with combined intensive immunosuppressive therapy. Recently, several additional therapies, including tofacitinib (TOF) and plasma exchange (PE) therapy, have been reported to be effective. We herein report a case of CADM-ILD with a high level of anti-MDA5 Ab that was refractory to combined intensive immunosuppressive therapy including TOF, but successfully treated with PE. The following are possible reasons why TOF was ineffective: (1) cytokines that were not suppressed by TOF played an important role in RP-ILD; (2) TOF was administered later than previously reported; and (3) TOF did not suppress pathological substances such as antibodies. On the other hand, PE removes cytokines and various pathological substances. Therefore, PE may be a more reasonable additional therapy for intractable CADM-ILD.