Rituximab resolves complications in anti-MDA5–positive juvenile dermatomyositis

Treatment with rituximab (RTX) in children with juvenile dermatomyositis (JDM) and antibodies to antimelanoma differentiation-associated gene 5 (anti-MDA5) results in clinical improvements in interstitial lung disease (ILD) as well as in cutaneous manifestation and musculoskeletal disease activity, according to a study.

The authors identified and assessed RTX-treated patients, aged 2‒21 years, who had been diagnosed with JDM, positive anti-MDA5 antibodies, and evidence of ILD at the Children’s Hospital at Montefiore between July 2012 and August 2021. They then reviewed the patients’ retrospective clinical and laboratory data.

Of the eight patients with positive anti-MDA5 antibodies, five (62.5 percent) had evidence of ILD, but only four had available data for review. All patients were treated with at least five courses of RTX infusions and discontinued steroids by about 12 months following RTX initiation. By the fifth course, participants had fewer than two concurrent medications.

Over the course of RTX treatment, all patients demonstrated either improved or fully resolved indicators of ILD on high-resolution computed tomography and pulmonary function tests. Active cutaneous manifestations and musculoskeletal disease activity also improved with RTX.

“Further studies are needed to better understand the efficacy of RTX for JDM disease-related complications,” the authors said.

Children with JDM and anti-MDA5 antibodies are at greater risk of severe disease complications, including ILD, according to the authors.