Rapidly progressive interstitial lung disease due to anti-melanoma differentiation associated protein-5 requiring a bilateral lung transplant, and complicated by kennel cough

Andrew R. Deitchman, Or Kalchiem-Dekel, Nevins Todd, Robert M. Reed

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

The association between inflammatory myopathies anti-synthetase syndrome and interstitial lung disease has been recognized since the 1950s. Patients generally present with gradual onset of symptoms and slow progression of fibrosis over months to years. Herein, we describe a previously well 51-year-old man who presented with three months of progressive small joint arthritis, cough, dyspnea, and eventually hypoxemic respiratory failure following a viral prodrome. He continued to decompensate despite high dose corticosteroids and mycophenolate mofetil, ultimately requiring extracorporeal membranous oxygenation as a bridge to bilateral lung transplantation. Clinically amyopathic dermatomyositis (CADM) was confirmed through serum positivity for anti-Melanoma Differentiation Associated Protein-5 (MDA-5) antibody. Interestingly, his post-operative course was complicated by a zoonotic infection with Bordetella bronchiseptica. This case highlights the importance of identifying rare autoimmune diseases, and the utility of transfer to a lung transplant center.

Original languageEnglish
Article number100886
JournalRespiratory Medicine Case Reports
Volume28
DOIs
StatePublished - 1 Jan 2019
Externally publishedYes

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