Longitudinal study of patients with antimelanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated interstitial lung disease
Authors' conclusion
MDA5+ DM-ILD patients had a high mortality rate despite aggressive treatment. Patients who survived the first year usually showed a significant improvement in serological markers and pulmonary function during the long-term follow-up.
Study context
- Population
- Patients diagnosed with anti-MDA5 antibody-positive DM-ILD70 analysed long-term
- Participants
- 216 patients
- Outcome
- Long-term survival rate
- Follow-up
- Up to 96 months
Methodological detailslongitudinal, 8-year follow-up
- Longitudinal
- 8-year follow-up
Main finding
As reported in the abstract
Mortality during follow-up
Patients who died during follow-up
85
39.3%
Observational data: associations, not effects.
Further findings
Timing of deaths · first year after diagnosis
- Share of deaths occurring in the first year after diagnosis
- 89%
Long-term serological and respiratory outcomes in first-year survivors · up to 96 months
- Long-term outcome reported in 70 patients
- Serological markers incl. anti-MDA5 antibody showed significant improvement with time
- Radiographic findings (HRCT scores) and pulmonary function (estimated FVC, estimated DLCO, dyspnoea scores) improved notably, especially in rapidly progressive ILD
- Early anti-fibrosis therapy helped to improve long-term pulmonary function
Original publication
Longitudinal study of patients with antimelanoma differentiation-associated gene 5 antibody-positive dermatomyositis-associated interstitial lung disease. Rheumatology (Oxford). Published 21 September 2022. doi:10.
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