Department of Respiratory and Critical Care Medicine, Zigong First People's Hospital, Zigong, Sichuan, China.
Int J Rheum Dis. 2023 Jun;26(6):1137-1142. doi: 10.1111/1756-185X.14575. Epub 2023 Jan 23.
Anti-MDA5 antibody dermatomyositis (DM) is a special type of myositis, which can potentially cause rapidly progressive interstitial lung disease (RP-ILD). Mixed connective tissue disease (MCTD) is a complex disease with different characteristics of autoimmune connective tissue disease, associated with ILD. Both are rare diseases, and few patients with both diseases have been reported. A 71-year-old woman complained of palpitations, with a 2 months history of rash around her hands, extensor surface of right elbow, and the nape of her neck. Subsequently, the patient had acute exacerbation of dyspnea and tachypnea. Anti-Ro52, U1 RNP and MDA5 antibodies were positive; the presenting evidence was suggestive of anti-MDA5 DM-RP-ILD complicated with MCTD. Our patient deteriorated rapidly and had a fatal outcome, despite "triple therapy" for RP-ILD. This case illustrates that patients with coexisting anti-MDA5 DM and MCTD have the former's typical clinical manifestations, and may develop ILD quickly rather than slowly as in MCTD, especially with the coexistence of anti-Ro52 antibodies.
抗 MDA5 抗体皮肌炎(DM)是一种特殊类型的肌炎,可能导致快速进展性间质性肺病(RP-ILD)。混合性结缔组织病(MCTD)是一种具有不同自身免疫性结缔组织病特征的复杂疾病,与ILD 相关。这两种疾病都很少见,很少有同时患有这两种疾病的患者报告。一名 71 岁女性因心悸就诊,其手部、右肘伸面和颈后有 2 个月的皮疹史。随后,患者出现呼吸困难和呼吸急促的急性加重。抗 Ro52、U1 RNP 和 MDA5 抗体阳性;临床表现提示抗 MDA5 DM-RP-ILD 合并 MCTD。尽管对 RP-ILD 进行了“三联疗法”,但我们的患者病情迅速恶化,最终导致死亡。该病例表明,同时患有抗 MDA5 DM 和 MCTD 的患者具有前者的典型临床表现,并且可能迅速而不是像 MCTD 那样缓慢发展为 ILD,尤其是在存在抗 Ro52 抗体的情况下。