Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.
Rheumatol Int. 2022 Jul;42(7):1271-1280. doi: 10.1007/s00296-021-04851-1. Epub 2021 Apr 10.
Although pregnancy is an important risk factor for autoimmune rheumatic diseases, little is known regarding the association between pregnancy and dermatomyositis (DM) or polymyositis (PM). Herein, we present two patients with DM that developed during the perinatal period. The first patient was positive for anti-aminoacyl synthetase (ARS) antibody and developed DM in the 14th week of pregnancy. Despite treatment, her foetus died of intrauterine growth restriction in the 27th week. The second patient was positive for anti-melanoma differentiation-associated gene 5 (MDA-5) antibody and developed DM 1 week after miscarriage at 9 weeks of gestation. The patient developed severe interstitial pneumonia, and intensive therapy including tofacitinib and rituximab administration was required. Our cases and a literature review revealed that various myositis-specific autoantibodies, including anti-ARS, anti-Mi-2, anti-TIF-1γ, and anti-MDA-5, are associated with DM and PM triggered by pregnancy. We also found that delay in commencing treatment in case of active disease including myositis and interstitial pneumonia, and poor response to corticosteroids were related to poor foetal outcomes in DM and PM. Although rare in pregnant women, it is critical to consider the possibility of DM and PM in patients presenting with rash, fever, weakness, and cough, and testing for myositis-specific autoantibodies is recommended.
虽然妊娠是自身免疫性风湿病的一个重要危险因素,但对于妊娠与皮肌炎(DM)或多发性肌炎(PM)之间的关系知之甚少。在此,我们介绍了两名围产期发生 DM 的患者。第一例患者抗氨酰基-tRNA 合成酶(ARS)抗体阳性,在妊娠第 14 周发生 DM。尽管进行了治疗,但她的胎儿在第 27 周因宫内生长受限而死亡。第二例患者抗黑色素瘤分化相关基因 5(MDA-5)抗体阳性,在妊娠 9 周时流产后 1 周发生 DM。患者发生严重间质性肺炎,需要进行包括托法替尼和利妥昔单抗在内的强化治疗。我们的病例和文献复习表明,包括抗-ARS、抗-Mi-2、抗-TIF-1γ和抗-MDA-5 在内的各种肌炎特异性自身抗体与妊娠诱发的 DM 和 PM 有关。我们还发现,包括肌炎和间质性肺炎在内的活动期疾病开始治疗的延迟以及对皮质类固醇反应不佳与 DM 和 PM 中的不良胎儿结局有关。尽管在孕妇中很少见,但对于出现皮疹、发热、乏力和咳嗽的患者,需要考虑 DM 和 PM 的可能性,并建议检测肌炎特异性自身抗体。