Effective Administration of Rituximab in Anti-MDA5 Antibody–Positive Dermatomyositis with Rapidly Progressive Interstitial Lung Disease and Refractory Cutaneous Involvement: A Case Report and Literature Review

Abstract

We describe the case of a 48-year-old man with dermatomyositis (DM) who demonstrated rapidly progressive interstitial lung disease (RP-ILD) and refractory cutaneous involvement together with high levels of anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab). Even after combination immunosuppressive therapy including a corticosteroid, cyclosporine A, and intravenous cyclophosphamide, his respiratory insufficiency and cutaneous involvement progressively worsened. However, the administration of rituximab (RTX) resulted in clinical remission as well as a visible reduction in anti-MDA5-Ab levels, suggesting that RTX could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those of RP-ILD related to anti-MDA5-Ab–positive DM.

Efficacy and safety of rituximab in the treatment of connective tissue disease-related interstitial lung disease

“…Recently, successful treatment of ILD related to anti-MDA5 IIM with RTX in addition to other immunosuppressive drugs was reported in some case reports. 60 , 61 In particular, RTX allowed the improvement of respiratory symptoms, HRCT findings, and lung function tests in 4 patients with anti-MDA5-related ILD refractory to GCs and immunosuppressants; of note, 2 patients developed infective adverse events within 6 months after RTX infusion. 62 On the other hand, in another case series, two elderly patients died despite aggressive immunosuppressive treatments including RTX.…”

Cyclophosphamide Versus Obinutuzumab for the Treatment of Anti-MDA5 Positive Inflammatory Myopathy with Interstitial Lung Disease: A Study Protocol and Literature Review

“…In addition, given vasculopathy is the main pathological finding of anti-MDA5 disease and the proven efficacy of anti-CD20 therapy in treating systemic vasculitides, anti-CD20 is a promising treatment option that might ameliorate the vascular inflammation and the subsequent intraparenchymal fibrosis in patients with RP-ILD [35]. Isolated case reports have described efficacy of RTX in anti-MDA5-associated RP-ILD [36,37]. We have recently reported four anti-MDA5 positive DM patients with refractory RP-ILD successfully treated by RTX [38].…”

Pharmacological Interventions for Pulmonary Involvement in Rheumatic Diseases

“…Moreover, a Spanish expert group agreed that anti-MDA5-positive ILD should be similarly treated [ 79 ]. Of note, despite the disease-specific anti-MDA5 autoantibody, successful treatment with rituximab has only been anecdotally reported [ 80 ].…”