Anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis: early detection is crucial.
BMJ Case Rep
; 17(6)2024 Jun 22.
Article
in En
| MEDLINE
| ID: mdl-38908836
ABSTRACT
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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Lung Diseases, Interstitial
/
Dermatomyositis
/
Interferon-Induced Helicase, IFIH1
Limits:
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
BMJ Case Rep
Year:
2024
Document type:
Article
Affiliation country:
Australia
Publication country:
ENGLAND
/
ESCOCIA
/
GB
/
GREAT BRITAIN
/
INGLATERRA
/
REINO UNIDO
/
SCOTLAND
/
UK
/
UNITED KINGDOM