Long-term survival of patients with idiopathic inflammatory myopathies: anatomy of a single-centre cohort
Authors' conclusion
IIM is a rare disease with important systemic complications.
Study context
- Population
- IIM patients fulfilling the Bohan and Peter criteria, single centre (mean age at diagnosis 40.8±15.6 years; 77.2% female)
- Participants
- 158 patients
- Exposure
- APM vs ADM vs Juvenile-onset dermatomyositis vs overlap' myositis (OM) vs Cancer-associated myositis vs Antisynthetase syndrome
- Outcome
- Mortality and survival; predictors of mortality
Methodological detailssingle-centre, Kaplan-Meier and Cox proportional hazards regression
- Single-centre
- Kaplan-Meier and Cox proportional hazards regression
Main finding
As reported in the abstract
Survival rates · 5, 10, 15, 20 and 25 years
- Survival at 5 years
- 89%
- Survival at 10 years
- 74%
- Survival at 15 years
- 67%
- Survival at 20 years
- 62%
- Survival at 25 years
- 43%
- Died during median follow-up of 13.6±10.2 years
- 29.1%
- Infection as most common cause of death
- 28.3%
Observational data: associations, not effects.
Further findings
Independent predictors of mortality
| HR (95% CI) | ||
|---|---|---|
| Older age at diagnosis | 1.053 (1.027–1.080) | |
| Cardiac involvement | 2.381 (1.237–4.584) | |
| Infections | 2.360 (1.194–4.661) | |
| 0.20.5125Hazard ratio (log scale) |
Diagnosis frequency
- ADM
- 35.4%
- OM
- 20.9%
- APM
- 24.7%
Organ involvement
- ILD
- 38.5%
- Gastrointestinal involvement
- 36.5%
- Cardiac involvement
- 23.4%
Treated with steroids plus one-to-three immunosuppressive drugs
- Proportion of patients
- 74.1%
Original publication
Long-term survival of patients with idiopathic inflammatory myopathies: anatomy of a single-centre cohort. Clin Exp Rheumatol. Published 1 March 2023. doi:10.
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