Objectives: Understanding health-related quality of life (HRQoL) is important in the management of patients with systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-APAH), however, little is known about HRQoL and its determinants in these patients.
Methods: A total of 60 female SLE-APAH patients with mean age of 33.5 years were prospectively recruited from May 2013 to November 2014. Right heart catheter, SF-36 generic questionnaire, disease activity and functional status were assessed in all patients.
Results: The median duration of SLE was 5 years. Thirty-five participants were with low disease activity (SLEDAI: 0-4). Patients with SLE-APAH reported significant impairment in HRQoL. The mean physical component summary (PCS) and mental component summary (MCS) scores were 46.4 and 56.9, respectively. Among haemodynamic measurements, higher pulmonary vascular resistance and lower cardiac output (CO) were associated with worse HRQoL. Lower body mass index (BMI), lower mean blood pressure and higher disease activity were also associated with poor HRQoL. Multivariate analysis revealed that lower SLEDAI and higher mean blood pressure were predictors for better PCS. However, higher CO (CO≥4L/min) was the only parameter independently associated with both better PCS and MCS.
Conclusions: Self-reported HRQoL was impaired in patients with SLE-APAH. Higher CO was the most important predictor for better HRQoL in these patients.