AB0161 early initiation of quadruple therapy in patients with anti-melanoma differentiation-associated 5 gene antibody-positive (MDA5) dermatomyositis (DM) with rapidly progressive interstitial lung disease (RP-ILD) – a retrospective case series
Abstract
MDA5 autoantibodies have been associated with an amyopathic form of dermatomyositis, with a subset developing RP-ILD with high mortality of 50%, despite intensive immunosuppressive therapies like high dose prednisolone, cyclophosphamide and calcineurin inhibitors.[1]Tofacitinib and rituximab have also been used as single agents, mainly as salvage therapy.[2]We aimed to investigate the role of early use of quadruple therapy (high dose steroids, one dose of cyclophosphamide, tofacitinib, rituximab) in patients with anti-MDA5 Ab-positive DM with RP-ILD.This is a retrospective case series involving 4 patients with anti-MDA5 Ab-positive DM with RP-ILD from 2019 to 2021. They were treated with quadruple therapy. Their clinical outcomes including survival and adverse events were evaluated.At initial diagnosis of RP-ILD, all patients were given pulsed methylprednisolone and 1 dose of cyclophosphamide. Tofacitinib was started within 2 weeks, followed by rituximab. 3 patients achieved clinical and biochemical improvement over time, with reduction of glucocorticoids by 6 months. Of the 4 patients, 1 required ICU stay and died. Severe adverse events were observed, most of which were infective complications.Table 1.Characteristics of patients with anti-MDA5 Ab-positive DM with RP-ILDCase 1Case 2Case 3Case 4Age/Gender60 F72 F48 M61 FClinical Presentation1stsymptom onset to RP-ILD4 weeks5 weeks4 weeks8 weeksSystemicFever, LOA, LOWFever, LOA, LOWFever, LOWFeverRespiratoryCough, hoarse…
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Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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