In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicentre study

Rheumatology (Oxford). 2023 Oct 3;62(10):3261-3267. doi: 10.1093/rheumatology/kead055.


Objective: Pulmonary arterial hypertension (PAH) is a leading cause of death in MCTD. We aimed to describe PAH in well-characterized MCTD patients.

Methods: MCTD patients enrolled in the French Pulmonary Hypertension Registry with a PAH diagnosis confirmed by right heart catheterization were included in the study and compared with matched controls: MCTD patients without PAH, SLE patients with PAH and SSc patients with PAH. Survival rates were estimated by the Kaplan-Meier method and risk factors for PAH in MCTD patients and risk factors for mortality in MCTD-PAH were sought using multivariate analyses.

Results: Thirty-six patients with MCTD-PAH were included in the study. Comparison with MCTD patients without PAH and multivariate analysis revealed that pericarditis, polyarthritis, thrombocytopenia, interstitial lung disease (ILD) and anti-Sm antibodies were independent predictive factors of PAH/PH in MCTD. Estimated survival rates at 1, 5 and 10 years following PAH diagnosis were 83%, 67% and 56%, respectively. MCTD-PAH presentation and survival did not differ from SLE-PAH and SSc-PAH. Multivariate analysis revealed that tobacco exposure was an independent factor predictive of mortality in MCTD-PAH.

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.

Keywords: MCTD; SLE; SSc; pulmonary arterial hypertension; survival.

Publication types

  • Multicenter Study

MeSH terms

  • Antibodies, Antinuclear
  • Familial Primary Pulmonary Hypertension
  • Humans
  • Lung Diseases, Interstitial* / etiology
  • Mixed Connective Tissue Disease* / complications
  • Pericarditis*
  • Pulmonary Arterial Hypertension*
  • Scleroderma, Systemic* / complications
  • Thrombocytopenia*


  • Antibodies, Antinuclear