학술논문

Fatal rapidly progressive interstitial lung disease in a patient with amyopathic dermatomyositis
Document Type
article
Source
Annals of Thoracic Medicine, Vol 16, Iss 3, Pp 294-298 (2021)
Subject
amyopathic dermatomyositis
connective tissue disease
idiopathic inflammatory myopathy
interstitial lung disease
Diseases of the circulatory (Cardiovascular) system
RC666-701
Diseases of the respiratory system
RC705-779
Language
English
ISSN
1817-1737
1998-3557
Abstract
A wide spectrum of interstitial lung disease (ID) is common and is a well-established manifestation of idiopathic inflammatory myopathies. Yet, till now, the pathogenetic mechanisms are still poorly understood, classification is evolving and prognosis is variable. A refractory and rapidly aggressive form of interstitial lung disease (RPILD) associated with dermatomyositis (DM) with minimal muscle weakness and normal creatine kinase (termed clinically amyopathic DM) is increasingly being recognized, with more incidence in Asians. However, we are not aware of reports of the Arab region. Herein, we present a 38-year-old male with this condition that ended with a fatal outcome despite aggressive therapy, with a review of recent literature.