III. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Department of Rheumatology and Immunology, Klinikum Bad Bramstedt, Bad Bramstedt, Germany.
Rheumatol Int. 2022 Dec;42(12):2267-2276. doi: 10.1007/s00296-022-05176-3. Epub 2022 Aug 8.
Dermatomyositis is a rare, type I interferon-driven autoimmune disease, which can affect muscle, skin and internal organs (especially the pulmonary system). In 2021, we have noted an increase in new-onset dermatomyositis compared to the years before the SARS-CoV-2 pandemic in our center. We present four cases of new-onset NXP2 and/or MDA5 positive dermatomyositis shortly after SARS-CoV-2 infection or vaccination. Three cases occurred within days after vaccination with Comirnaty and one case after SARS-CoV-2 infection. All patients required intensive immunosuppressive treatment. MDA5 antibodies could be detected in three patients and NXP2 antibodies were found in two patients (one patient was positive for both antibodies). In this case-based systematic review, we further analyze and discuss the literature on SARS-CoV-2 and associated dermatomyositis. In the literature, sixteen reports (with a total of seventeen patients) of new-onset dermatomyositis in association with a SARS-CoV-2 infection or vaccination were identified. Ten cases occurred after infection and seven after vaccination. All vaccination-associated cases were seen in mRNA vaccines. The reported antibodies included for instance MDA5, NXP2, Mi-2 and TIF1γ. The reviewed literature and our cases suggest that SARS-CoV-2 infection and vaccination may be considered as a potential trigger of interferon-pathway. Consequently, this might serve as a stimulus for the production of dermatomyositis-specific autoantibodies like MDA5 and NXP2 which are closely related to viral defense or viral RNA interaction supporting the concept of infection and vaccination associated dermatomyositis.
皮肌炎是一种罕见的 I 型干扰素驱动的自身免疫性疾病,可影响肌肉、皮肤和内脏器官(特别是肺部系统)。2021 年,与 SARS-CoV-2 大流行之前相比,我们中心注意到新发皮肌炎的病例有所增加。我们报告了四例新诊断的 NXP2 和/或 MDA5 阳性皮肌炎,这些患者在 SARS-CoV-2 感染或接种疫苗后不久发病。三例发生在接种 Comirnaty 后几天内,一例发生在 SARS-CoV-2 感染后。所有患者均需要强化免疫抑制治疗。三例患者可检测到 MDA5 抗体,两例患者可检测到 NXP2 抗体(一例患者两种抗体均为阳性)。在本基于病例的系统回顾中,我们进一步分析和讨论了 SARS-CoV-2 与皮肌炎相关的文献。文献中,共发现 16 例(共 17 例患者)新发皮肌炎与 SARS-CoV-2 感染或接种相关的病例报告。10 例发生在感染后,7 例发生在接种后。所有与接种相关的病例均发生在 mRNA 疫苗接种后。报道的抗体包括 MDA5、NXP2、Mi-2 和 TIF1γ 等。回顾的文献和我们的病例表明,SARS-CoV-2 感染和接种可能被认为是干扰素途径的潜在触发因素。因此,这可能会刺激产生皮肌炎特异性自身抗体,如 MDA5 和 NXP2,这些抗体与病毒防御或病毒 RNA 相互作用密切相关,支持感染和接种相关皮肌炎的概念。