Rapidly progressive interstitial lung disease in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis: serial changes on HRCT.
Anti-melanoma differentiation
HRCT
Interstitial lung disease
Journal
Emergency radiology
ISSN: 1438-1435
Titre abrégé: Emerg Radiol
Pays: United States
ID NLM: 9431227
Informations de publication
Date de publication:
Dec 2022
Dec 2022
Historique:
received:
05
05
2022
accepted:
25
07
2022
pubmed:
3
8
2022
medline:
11
11
2022
entrez:
2
8
2022
Statut:
ppublish
Résumé
Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies in patients with dermatomyositis are associated with rapidly progressive interstitial lung disease (RP-ILD). Computed tomography (CT) plays a central role in the diagnosis of RP-ILD and may help characterize the temporal changes. We report five anti-MDA5-positive dermatomyositis patients with serial CT scans spanning their acute RP-ILD disease course. Our case series highlights the variable imaging pattern that can manifest in this setting, including diffuse alveolar damage and nonspecific interstitial pneumonia patterns. Three patients in our series died within 4 months of their disease onset, whereas the other two patients survived. The serial CT changes in anti-MDA5 disease are dynamic and variable; therefore, it is imperative to maintain a broad differential when faced with these HRCT patterns to improve the diagnosis and management of this underrecognized entity.
Sections du résumé
BACKGROUND
BACKGROUND
Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies in patients with dermatomyositis are associated with rapidly progressive interstitial lung disease (RP-ILD). Computed tomography (CT) plays a central role in the diagnosis of RP-ILD and may help characterize the temporal changes.
METHODS
METHODS
We report five anti-MDA5-positive dermatomyositis patients with serial CT scans spanning their acute RP-ILD disease course.
RESULTS
RESULTS
Our case series highlights the variable imaging pattern that can manifest in this setting, including diffuse alveolar damage and nonspecific interstitial pneumonia patterns. Three patients in our series died within 4 months of their disease onset, whereas the other two patients survived.
CONCLUSION
CONCLUSIONS
The serial CT changes in anti-MDA5 disease are dynamic and variable; therefore, it is imperative to maintain a broad differential when faced with these HRCT patterns to improve the diagnosis and management of this underrecognized entity.
Identifiants
pubmed: 35918568
doi: 10.1007/s10140-022-02080-y
pii: 10.1007/s10140-022-02080-y
doi:
Substances chimiques
Interferon-Induced Helicase, IFIH1
EC 3.6.4.13
Autoantibodies
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
961-967Subventions
Organisme : NIAMS NIH HHS
ID : K23 AR075898
Pays : United States
Informations de copyright
© 2022. The Author(s), under exclusive licence to American Society of Emergency Radiology (ASER).
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