Marked Long‐Term Improvement in Lung Function in Melanoma Differentiation–Associated Protein 5 Antibody–Positive Dermatomyositis Patients: Experience of a Single‐Center Longitudinal Cohort in North America
Authors' conclusion
In a North American MDA5 antibody-associated DM-ILD cohort treated with aggressive combination immunomodulatory therapy including predominantly mycophenolate, IVIg, and rituximab, disease mortality was low and lung function improved markedly.
Study context
- Population
- North American patients with MDA5 antibody-associated dermatomyositis (DM), single-centre longitudinal cohort of 352 patients with idiopathic inflammatory myopathies24 with interstitial lung disease (ILD)
- Participants
- 30 patients
- Outcome
- Longitudinal disease course and pulmonary outcomes (pulmonary function tests (PFT), high-resolution CT chest)
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- ILD
- 80% (n = 24/30)
- Mortality in ILD at mean ± SD follow-up of 4.0 ± 0.8 years
- 2/24
- Improvement in % predicted forced vital capacity (FVC) at 1.8 ± 0.6 years (18 of 22 surviving ILD patients)
- 16%
- Improvement in % predicted DLCO at 1.8 ± 0.6 years (18 of 22 surviving ILD patients)
- 17%
- Improvement in % predicted FVC at long-term follow-up, mean ± SD 6.8 ± 3.4 years (10 of 18)
- 24%
- Improvement in % predicted DLCO at long-term follow-up, mean ± SD 6.8 ± 3.4 years (10 of 18)
- 20%
Observational data: associations, not effects.
Further findings
Therapies at follow-up · 4.0 ± 0.8 years
- Number of therapies (mean ± SD)
- 3.1 ± 1.3
- Intravenous Ig (IVIg)
- 79%
- RTX
- 58%
- Mycophenolate
- 67%
- GC
- 63%
Biomarkers
- MDA5 antibody and IL-15 levels and PON1 enzyme activity correlated significantly with disease activity at baseline and longitudinally
Original publication
Marked Long‐Term Improvement in Lung Function in Melanoma Differentiation–Associated Protein 5 Antibody–Positive Dermatomyositis Patients: Experience of a Single‐Center Longitudinal Cohort in North America. Arthritis Care Res (Hoboken). Published 24 March 2026. doi:10.
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