Abstract
BACKGROUND: Clinically, amyopathic dermatomyositis is a clinically distinct subgroup of dermatomyositis characterised by unique dermatological manifestations without muscle involvement. Clinically, amyopathic dermatomyositis is frequently associated with interstitial lung disease, which usually has a rapidly progressive, fatal clinical course. Although clinically, amyopathic dermatomyositis-related interstitial lung disease is well described, data on the histopathology of clinically, amyopathic dermatomyositis-interstitial lung disease are limited. Organising pneumonia and pulmonary vasculitis have rarely been reported. CASE REPORT: A 54-year-old Korean woman presented with exertional dyspnoea and a dry cough. Chest computed tomography revealed subpleural ground-glass opacities suggesting interstitial lung disease, which was later pathologically confirmed to be a combination of organising pneumonia and pulmonary vasculitis. The patient improved markedly with prednisone treatment and has remained stable for a long time. CONCLUSION: We hereby report a rare combination of organising pneumonia and pulmonary vasculitis in a patient with amyopathic dermatomyositis-interstitial lung disease.