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抗 MDA5 抗体相关性皮肌炎相关快速进展性间质性肺病合并混合性结缔组织病患者:一例报告。

Anti-MDA5 antibody dermatomyositis-associated rapidly progressive interstitial lung disease patient complicated with mixed connective tissue disease: A case report.

机构信息

Department of Respiratory and Critical Care Medicine, Zigong First People's Hospital, Zigong, Sichuan, China.

出版信息

Int J Rheum Dis. 2023 Jun;26(6):1137-1142. doi: 10.1111/1756-185X.14575. Epub 2023 Jan 23.

Abstract

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.

摘要

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。

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