Sjögren's disease & overlap diseases
In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicentre study
Authors' conclusion
PAH is a rare and severe complication of MCTD associated with a 56% 10-year survival. We identified ILD, pericarditis, thrombocytopenia and anti-Sm antibodies as risk factors for PAH in MCTD and tobacco exposure as a predictor of mortality in MCTD-PAH.
Study context
- Population
- MCTD patients with right heart catheterization-confirmed pulmonary arterial hypertension (PAH) enrolled in the French Pulmonary Hypertension Registry
- Participants
- 36 MCTD-PAH patients
- Exposure
- MCTD-PAH vs matched controls: MCTD without PAH, SLE-PAH and SSc-PAH
- MCTD-PAHn = 36
- Outcome
- Survival after PAH diagnosis in MCTD-PAH (Kaplan-Meier)
- Follow-up
- 1, 5 and 10 years
Methodological detailsnational multicentre, matched controls, registry-based, multivariate analysis, Kaplan-Meier survival
- National multicentre
- Matched controls
- Registry-based
- Multivariate analysis
- Kaplan-Meier survival
Main finding
As reported in the abstract
| Group | 1-year survival | 5-year survival | 10-year survival |
|---|---|---|---|
| MCTD-PAH | 83% | 67% | 56% |
MCTD-PAH
1-year survival83%
5-year survival67%
10-year survival56%
Observational data: associations, not effects.
Further findings
Independent predictive factors of PAH/PH in MCTD (vs MCTD without PAH, multivariate)
- Pericarditis, polyarthritis, thrombocytopenia, interstitial lung disease (ILD) and anti-Sm antibodies were independent predictive factors
Presentation and survival vs SLE-PAH and SSc-PAH
- MCTD-PAH presentation and survival did not differ from SLE-PAH and SSc-PAH
Predictors of mortality in MCTD-PAH (multivariate)
- Tobacco exposure was an independent predictor of mortality
Original publication
In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicentre study. Rheumatology (Oxford). Published 2 February 2023. doi:10.
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