A 10-year Analysis of Survival in Pulmonary Arterial Hypertension With Versus Without Associated Connective Tissue Disease

Although patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (PAH) have a higher risk of death than the overall PAH population, recent improvements in treatments and therapies have led to reduced morbidity/mortality risks for both groups over the last 10 years, thereby increasing survival rates, according to a report.

“Availability of new and combination therapy approaches targeting multiple pathophysiologic pathways have led to improved outcomes in PAH. However, trials of PAH therapies generally enroll patients with different etiologies of PAH, and trials devoted solely to those with CTD-PAH are rare; therefore, the magnitude of treatment effect in CTD-PAH is poorly defined, as these patients represent a subgroup in most trials, albeit a large one,” the researchers explained.

They queried PubMed and Embase for randomized, controlled trials (RCTs) or registries from 2000 to 2019 with at least 30 patients. RCT eligibility criteria were evaluation of an approved PAH therapy and long-term morbidity/mortality risks or six-minute walk distance. Registry eligibility criteria included survival rates.

Collectively, there were 11 RCTs encompassing 4,329 total patients, of whom 1,267 had CTD-PAH. There were also 19 registries encompassing 9,739 total patients, of whom 4,008 had CTD-PAH. Compared to controls, there were similar reductions in risks of clinical morbidity/mortality due to investigational therapy in the overall PAH group (hazard ratio [HR], 0.64; 95% confidence interval [CI], 0.54-0.75; P<0.001) and the CTD-PAH group (HR, 0.64; 95% CI, 0.51-0.81; P<0.001).

The CTD-PAH group, compared to all patients with PAH, had a lower three-year survival rate (62% [95% CI, 57-67] vs 72% [95% CI, 69-75]). However, patients with CTD-PAH treated mainly after 2010, compared to those treated before 2010, had a higher three-year survival rate (73% [95% CI, 62-81] vs 65% [95% CI, 59-71]).

The study was published in Arthritis & Rheumatology.

“Patients with CTD-PAH have a higher risk of death than the overall PAH population; however, survival has improved among this subgroup treated in the last 10 years compared to earlier cohorts. Patients with systemic sclerosis have worse survival rates than those with systemic lupus erythematosus. Given the high risk of mortality in these patients, early detection and up-front aggressive treatment are warranted,” the researchers stated in their conclusion.