Aggressive multi‐combination therapy for anti‐MDA5 antibody‐positive dermatomyositis‐rapidly progressive interstitial lung disease

Abstract

AbstractObjectivesTo elucidate the efficacy and safety of aggressive multi‐combination therapy with mycophenolate mofetil, rituximab, and plasma exchange or polymyxin B immobilized fiber column direct hemoperfusion followed by conventional therapy with corticosteroids, calcineurin inhibitors, and intravenous pulse cyclophosphamide in patients with rapidly progressive interstitial lung disease (RPILD) with anti‐melanoma differentiation‐associated gene 5 (MDA5)‐antibody‐positive dermatomyositis (DM).MethodsA total of 23 patients with anti‐MDA5 antibody‐positive DM‐RPILD were enrolled, with nine patients in Group A (treated conventionally before March 2015) and 14 patients in Group B (received aggressive treatment after April 2015).ResultsPretreatment severity of interstitial lung disease (ILD) did not differ between the two groups. However, Group B exhibited a higher cumulative survival rate at 48 weeks than Group A (64.3% vs. 33.3%). The corticosteroid dose, divided by the initial dose at 3 months and 12 months, was significantly lower in Group B than in Group A (p = .046 and .026, respectively). Among the ILD‐related deaths in Group B, there was a tendency toward a higher proportion of males and more severe ILD. The incidence of infection did not differ between the groups, but leukopenia was more common in Group B.ConclusionThis aggressive multi‐combination therapy may improve the survival outcome of patients with anti‐MDA5 antibody‐positive DM‐RPILD. However, careful management of complications, such as opportunistic infections and leukopenia, is essential. Future refinement through longitudinal investigations tracking the long‐term efficacy, safety, and cost‐effectiveness of this treatment strategy is needed.