Sex-specific differences and survival in patients with idiopathic pulmonary arterial hypertension 2008-2016

Research output: Contribution to journalArticle


Background: Women with idiopathic pulmonary arterial hypertension (IPAH) have been found to have a worse haemodynamic status at diagnosis, but better survival than men. Over the past decade, demographics have changed and new treatments have become available. The objective of this study was to investigate sex differences in an incident IPAH population diagnosed between 2008 and 2016.

Methods: Differences in clinical characteristics of patients included in the Swedish Pulmonary Arterial Hypertension Register (SPAHR) were analysed at the time of diagnosis. Survival by sex was investigated using Cox proportional hazard regression and Kaplan-Meier curves.

Results: The study included 271 patients diagnosed with IPAH, median age was 68 (1st-3rd quartiles 54-74) years and 56% were women. At diagnosis, women were younger, had lower pulmonary vascular resistance and fewer comorbidities and more often received a combination of PAH-targeted therapies than men. Men had worse survival rates than women (hazard ratio 1.49; CI 1.02-2.18; p=0.038), but this difference did not remain after adjustment for age (hazard ratio 1.30; CI 0.89-1.90; p=0.178).

Conclusions: Men with incident IPAH have worse crude survival than women. This is due to women being younger with a less pronounced comorbidity burden than men at the time of diagnosis.


  • SveFPH
External organisations
  • Skåne University Hospital
  • Karolinska Institutet
  • Linköping University
  • University of Gothenburg
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Clinical Medicine
Original languageEnglish
JournalERJ Open Research
Issue number3
Publication statusPublished - 2019 Jul
Publication categoryResearch