Anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis: early detection is crucial

被引：0

作者：

Goradia, Shruti ^{[1
,2
]}

Holper, Sarah ^{[3
,4
]}

Lim, Yuan Z. ^{[5
,6
]}

Brady, Sharmayne R. E. ^{[6
]}

机构：

[1] Univ Melbourne, Fac Med Dent & Hlth Sci, Melbourne Med Sch, Melbourne, VIC, Australia

[2] Alfred Hosp, Dept Pharm, Melbourne, VIC, Australia

[3] Royal Melbourne Hosp, Dept Med, Parkville, VIC, Australia

[4] Walter & Eliza Hall, Immunol Div, Inst Med Res, Melbourne, VIC, Australia

[5] Monash Univ, Sch Publ Hlth & Prevent Med, Melbourne, VIC, Australia

[6] Alfred Hlth, Dept Rheumatol, Melbourne, VIC, Australia

来源：

BMJ CASE REPORTS | 2024年 / 17卷 / 06期

关键词：

medical management; connective tissue disease; interstitial lung disease; adult intensive care; INTERSTITIAL LUNG-DISEASE;

D O I：

10.1136/bcr-2023-259338

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Anti-melanoma differentiation-associated gene 5-positive (Anti-MDA5) dermatomyositis (DM) is an aggressive phenotype of DM associated with rapidly progressive interstitial lung disease (RP-ILD). It is a rare condition that carries high mortality. Diagnosis and management of patients with anti-MDA5 DM RP-ILD presents several challenges, including uncertainty around treatment algorithms and a lack of evidence to inform practice. This case report of a patient with anti-MDA5 DM RP-ILD highlights these challenges, emphasising the fulminant course of this disease despite aggressive immunosuppression. Further research is required to guide management and to minimise morbidity and mortality, and greater awareness of the condition is required to minimise delays in diagnosis.

引用

页数：4

共 18 条

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