Inflammatory myopathies

Identification of Three Different Phenotypes in Anti–Melanoma Differentiation–Associated Gene 5 Antibody–Positive Dermatomyositis Patients: Implications for Prediction of Rapidly Progressive Interstitial Lung Disease

Arthritis RheumatolPublished Retrospective cohort study

Authors' conclusion

Cluster analysis identified 3 distinct clinical patterns and outcomes in our large cohort of anti-MDA5+ DM patients.

Study context

Population
Patients with anti-MDA5+ dermatomyositis (DM)retrospectively enrolled
Participants
265 patients
Exposure
Three phenotype clusters of anti-MDA5+ DM
  • Cluster 1n = 108
  • Cluster 2n = 72
  • Cluster 3n = 85
Outcome
Cumulative incidence of non-RPILD
Methodological detailsunsupervised hierarchical cluster analysis, decision tree analysis, external validation cohort
  • Unsupervised hierarchical cluster analysis
  • Decision tree analysis
  • External validation cohort

Main finding

As reported in the abstract

Observational data: associations, not effects.

Further findings

All-cause mortality — Cluster 3 vs Cluster 2 · across clusters 3, 2 and 1

P < 0.0001

Decision tree classification algorithm (8 variables) accuracy

Accuracy, development cohort
78.5%
Accuracy, external validation cohort
70.0%
  • Variables: age >50 years, disease course <3 months, myasthenia (proximal muscle weakness), arthritis, CRP, creatine kinase, anti-Ro 52 titer, anti-MDA5 titer

Original publication

Identification of Three Different Phenotypes in Anti–Melanoma Differentiation–Associated Gene 5 Antibody–Positive Dermatomyositis Patients: Implications for Prediction of Rapidly Progressive Interstitial Lung Disease. Arthritis Rheumatol. Published 17 March 2023. doi:10.1002/art.42308

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