Prevalence of pulmonary arterial hypertension in persons with connective tissue diseases, medication use patterns, and health care utilization.

摘要

The purpose of this study was to determine the prevalence of pulmonary arterial hypertension in a population with connective tissue diseases, to describe demographics and clinical characteristics of patients with pulmonary arterial hypertension associated with connective tissue diseases, to determine patterns of pulmonary arterial hypertension-specific medication use in patients with pulmonary arterial hypertension associated with connective tissue diseases, and to compare health care utilization pre-initiation and post-initiation of sildenafil in patients with pulmonary arterial hypertension associated with connective tissue diseases.;An observational database analysis was conducted using a Commercial Claims database. One year-prevalence of PAH among individuals with connective tissue diseases was calculated by dividing the number of individuals with PAH in 2009 by the total number of individuals with connective tissue diseases. Prevalence of use of pulmonary arterial hypertension-specific medication was determined for each individual medication and for each therapeutic class. Medication persistence for pulmonary arterial hypertension-specific medications was calculated by counting number of continuous days on the first pulmonary arterial hypertension-specific medication therapy during twelve months after initiation of therapy. A zero inflated negative binomial model with random effects was used to compare days in hospital per month pre-initiation and post-initiation of sildenafil.;The estimated prevalence of pulmonary arterial hypertension in individuals with connective tissue diseases was 0.92 percent (CI: 0.86% to 0.98%) in this general population. The prevalence of pulmonary arterial hypertension-specific medication use was 24.1 percent (95% CI: 21.9% to 26.4%). The most frequently prescribed pulmonary arterial hypertension-specific therapeutic classes were the phosphodiesterase-5 inhibitors (14.8%) and endothelin receptor antagonists (14.7%). The most frequently prescribed pulmonary arterial hypertension-specific medications were sildenafil (14%) and bosentan (11.5%). When not using sildenafil, for one unit increase in month, the expected number of days in hospital increased by 2.8 percent. When using sildenafil, for one unit increase in month, the expected number of days in hospital decreased by 3.51 percent.;The prevalence of pulmonary arterial hypertension in persons with connective tissue diseases was higher than prevalence of idiopathic pulmonary arterial hypertension. The current finding adds epidemiological evidence to better understand pulmonary arterial hypertension in connective tissue diseases and underscores the need for monitoring for pulmonary arterial hypertension in patients with connective tissue disease. Only 24% of the studied population was taking pulmonary arterial hypertension-specific medications. Analysis showed some evidence that the most frequently used medication in that group, Sildenafil, may reduce hospital days within the time interval studied.