دورية أكاديمية

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

التفاصيل البيبلوغرافية
العنوان: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis: early detection is crucial.
المؤلفون: Goradia S; Faculty of Medicine Dentistry and Health Sciences, The University of Melbourne, Melbourne Medical School, Melbourne, Victoria, Australia sgoradia@student.unimelb.edu.au.; Department of Pharmacy, Alfred Hospital, Melbourne, Victoria, Australia., Holper S; Department of Medicine, The Royal Melbourne Hospital, Parkville, Victoria, Australia.; Institute of Medical Research Immunology Division, Walter and Eliza Hall, Melbourne, Victoria, Australia., Lim YZ; Monash University School of Public Health and Preventive Medicine, Melbourne, Victoria, Australia.; Department of Rheumatology, Alfred Health, Melbourne, Victoria, Australia., Brady SRE; Department of Rheumatology, Alfred Health, Melbourne, Victoria, Australia.
المصدر: BMJ case reports [BMJ Case Rep] 2024 Jun 22; Vol. 17 (6). Date of Electronic Publication: 2024 Jun 22.
نوع المنشور: Case Reports; Journal Article
اللغة: English
بيانات الدورية: Publisher: BMJ Pub. Group Country of Publication: England NLM ID: 101526291 Publication Model: Electronic Cited Medium: Internet ISSN: 1757-790X (Electronic) Linking ISSN: 1757790X NLM ISO Abbreviation: BMJ Case Rep Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : BMJ Pub. Group
مواضيع طبية MeSH: Dermatomyositis*/diagnosis , Dermatomyositis*/immunology , Dermatomyositis*/complications , Interferon-Induced Helicase, IFIH1*/immunology , Lung Diseases, Interstitial*/diagnosis, Humans ; Autoantibodies/blood ; Early Diagnosis ; Fatal Outcome ; Male ; Female ; Middle Aged
مستخلص: 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.
Competing Interests: Competing interests: None declared.
(© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)
فهرسة مساهمة: Keywords: adult intensive care; connective tissue disease; interstitial lung disease; medical management
المشرفين على المادة: EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1)
EC 3.6.1.- (IFIH1 protein, human)
0 (Autoantibodies)
تواريخ الأحداث: Date Created: 20240622 Date Completed: 20240622 Latest Revision: 20240711
رمز التحديث: 20240711
DOI: 10.1136/bcr-2023-259338
PMID: 38908836
قاعدة البيانات: MEDLINE
الوصف
تدمد:1757-790X
DOI:10.1136/bcr-2023-259338