Inflammatory myopathies

Clinical, histologic and prognostic features of clinically amyopathic dermatomyositis

Clin Exp RheumatolPublished Retrospective cohort study

Authors' conclusion

CADM patients differ in disease onset, autoantibody profiles, joint and lung involvement. While laboratory and instrumental tests have not shown muscle involvement in CADM, many muscle biopsies have shown MHC-I overexpression.

Study context

Population
Patients of a DM cohort
Participants
63 / 12 / 16
Exposure
Clinical amyopathic dermatomyositis (CADM) vs classic DM vs hypomyopathic DM (HDM)
  • DMn = 63reference
  • HDMn = 12
  • CADMn = 16
Outcome
Features of CADM vs DM
Methodological detailsCox analysis
  • Cox analysis

Main finding

As reported in the abstract

Age at onset, years: CADM 45.5±17 vs DM 57±18
p<0.05
Age at diagnosis, years: CADM 46±17 vs DM 58±18
p<0.05
Anti-MDA5 positive: CADM 37.5% vs DM 4.8%
p<0.05
Anti-TIF1-γ positive: CADM 31.3% vs DM 6.3%
p<0.05
Arthritis: CADM 37.5% vs DM 12.6%
p<0.05
Interstitial lung disease (ILD): CADM 43.8% vs DM 15.9%
p<0.05

Observational data: associations, not effects.

Further findings

Five-year survival · 5 years

Mortality predictors, overall cohort (Cox analysis)

HR (CI)
ILD3.57 (1.11–11.5)
Cancer3.67 (1.17–11.5)
OutcomeHR (CI)
Mortality predictors, overall cohort (Cox analysis)CADMvs DMNot a mortality predictor1.46 (0.33–6.68)
OutcomeDMHDMCADM
Sarcolemma MHC-I expression on muscle biopsy
Biopsies available44117

Sarcolemma MHC-I expression on muscle biopsy

Similar sarcolemma MHC-I expression rates

Five-year survival · 5 years

Comparable across groups

Original publication

Clinical, histologic and prognostic features of clinically amyopathic dermatomyositis. Clin Exp Rheumatol. Published 14 March 2024. doi:10.55563/clinexprheumatol/kgpnbq

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