Rapidly progressive interstitial lung disease due to anti-melanoma differentiation associated protein-5 requiring a bilateral lung transplant, and complicated by kennel cough. (2019)

• Record Type: Journal Article
• Authors: Deitchman, Andrew R.; Kalchiem-Dekel, Or; Todd, Nevins; Reed, Robert M.
• Is Part Of: Respiratory medicine case reports. Volume 28(2019)
• Publication Date: 2019
• Subjects: Respiratory organs -- Diseases -- Periodicals; Chest -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Respiratory System -- Periodicals; Chest -- Diseases; Respiratory organs -- Diseases; Electronic journals; Periodicals; 616.2
• DOI: 10.1016/j.rmcr.2019.100886
• Languages: English
• ISSNs: 2213-0071

Abstract

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. Highlights: MDA-5 antibodies are a marker for poor prognosis in anti-synthetase syndrome. Early aggressive therapy should be strongly considered for rapidly-progressive ILD secondary to CADM. Early referral to a lung transplant facility may be potentially lifesaving. Zoonotic infections in transplant recipients are rare but clinically important pathogens.