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Background While sex differences characterize susceptibility and severity of idiopathic pulmonary arterial hypertension (IPAH), our understanding of the relationship between levels of gonadotropins and sex hormones in fertile women and the disease is limited. We aimed to investigate whether gonadotropin and sex hormone levels in women of reproductive age were associated with risk and mortality of IPAH. Methods We did a matched case-control study. Cases were reproductive female patients with idiopathic pulmonary arterial hypertension admitted in Shanghai Pulmonary Hospital (Tongji University School of Medicine, Shanghai, China) during 2008–2014. Healthy controls were matched on age and body mass index. We also did a prospective cohort study to assess the effects of hormone levels on mortality in IPAH fertile female patients. Results One hundred sixty-four cases and 133 controls were included. After adjustment for age and body mass index, the odds ratios of having IPAH for follicle-stimulating hormone, testosterone, and progesterone as expressed on natural log scale were 1.51 (95% confidence interval: 1.06, 2.16), 0.42 (0.31–0.57), and 0.52 (0.43–0.63), respectively. In the cohort study with a median follow-up of 77 months, the hazard ratios for dying after adjustment for baseline characteristics and treatments among IPAH patients were 2.01 (95% confidence interval: 1.22–3.30) and 0.78 (95% confidence interval: 0.62–0.98) for follicle-stimulating hormone and progesterone in natural log scale, respectively. Conclusions In reproductive women with IPAH, high follicle-stimulating hormone and low progesterone tended to be associated with high risk of IPAH and mortality among patients.

Association Between High FSH, Low Progesterone, and Idiopathic Pulmonary Arterial Hypertension in Women of Reproductive Age