Clinical Improvement in Early-Onset Interstitial Lung Disease Using Rituximab in Children With Antimelanoma Differentiation-Associated Gene 5–Positive Juvenile Dermatomyositis
Authors' conclusion
To our knowledge, this is the first study to examine the use of RTX in children with JDM and anti-MDA5 antibodies, with notable improvements in ILD, cutaneous, and musculoskeletal manifestations.
Study context
- Population
- Patients aged 2–21 years with juvenile dermatomyositis (JDM), positive anti-MDA5 antibodies and interstitial lung disease (ILD) treated with rituximab (RTX), Children's Hospital at Montefiore, July 2012–August 2021data available for review
- Participants
- 4 patients
- Exposure
- Disease course before vs after RTX
- RTXn = 4
- Outcome
- ILD indicators on high-resolution CT and pulmonary function tests
- Follow-up
- Over the course of RTX treatment
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
over the course of RTX treatment
- ILD indicators improved or fully resolved for all patients
- Steroids discontinued by an average of 12 months after starting RTX
- Decrease to fewer than 2 concurrent medications by the fifth course of RTX
Observational data: associations, not effects.
Further findings
ILD among anti-MDA5-positive patients (prevalence)
- Patients with positive anti-MDA5 antibodies with evidence of ILD: 5 of 8
- 62.5%
Steroid discontinuation
- Discontinuation of steroids by an average of 12 months after starting RTX
Cutaneous and musculoskeletal disease activity
- Resolution of active cutaneous manifestations and musculoskeletal disease activity
Original publication
Clinical Improvement in Early-Onset Interstitial Lung Disease Using Rituximab in Children With Antimelanoma Differentiation-Associated Gene 5–Positive Juvenile Dermatomyositis. J Rheumatol. Published 15 September 2023. doi:10.
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