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

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.

Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features

“…Shoji et al reported the case of a female patient with a rapidly progressive ILD associated with anti-MDA-5 antibodies that underwent successful lung transplantation after an intensive immunosuppressive therapy including cyclophosphamide and intravenous immunoglobulins (17). Other similar cases have been reported since then, with favorable outcomes (18,19). Like our two patients, at least one of them had anti-SSA 52 antibodies (18), which are frequently associated with anti-MDA5 antibodies (10) and seem to be correlated with severe forms of ILD (2,20).…”

Case Report: Rapidly Progressive Interstitial Lung Disease in A Pregnant Patient With Anti-Melanoma Differentiation-Associated Gene 5 Antibody-Positive Dermatomyositis

“…RP-ILD was mainly defined as progressive dyspnea, or according to HRCT findings secondary to ILD within 3 months after the onset of respiratory symptoms (9, 10). RP-ILD often occurs as a complication of CADM, which usually portends poor prognosis with reported mortality rates in the first year as high as 60% (11). Anti-MDA5 antibodies were first identified by Sato et al in 2005 in Japanese patients with CADM (12).…”

Pharmacologic Treatment of Anti-MDA5 Rapidly Progressive Interstitial Lung Disease

“…There is an additional experience of lung transplants reported by other authors [ 67 , 68 , 69 •, 70 , 74 ] including a total of 7 patients, all of them survived. Thus, lung transplantation is a viable therapeutic option in refractory patients with anti-MDA5-associated RP-ILD.…”

“…Thus, it applies to anti-MDA5-positive CADM-associated RP-ILD patients refractory to combined immunosuppressive therapy, mostly as a bridge to a most definitive solution like lung transplantation or while waiting for a response to the immunosuppressive drugs. Currently, there is scarce data on the efficacy of VV-ECMO in this type of patients [ 61 , 62 •, 63 – 68 , 69 •, 70 ], and was considered successful only in a few patients, either because it helped to improve the clinical status of the patient or it allowed performing a lung transplantation [ 67 , 68 , 69 •, 70 ].…”