Anti-MDA5 antibody-positive dermatomyositis with rapidly progressive interstitial lung disease: report of two cases. Issue 4 (28th April 2021)

• Record Type: Journal Article
• Authors: Mehta, Asmita A; Paul, Tisa; CB, Mithun; Haridas, Nithya
• Is Part Of: BMJ case reports. Volume 14:Issue 4(2021)
• Publication Date: 2021-04-28
• Subjects: respiratory system -- interstitial lung disease -- lung function; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2020-240046
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : Melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (DM) displays unique cutaneous and pathologic features. We describe two cases of myositis-associated rapidly progressive interstitial lung disease (RP-ILD). The patients were two women from Kerala, India. Both patients had anti-MDA5 antibody-positive myositis. Both patients presented with RP-ILD without any clinical features of myositis and succumbed to their illness despite aggressive medical treatment. Anti-MDA5-antibody-positive DM is characterised by amyopathic disease with rapidly progressive and fatal ILD.