Short Survival of Only 4 Years Still Characterizes Systemic Sclerosis Patients with PAH, Australian Study Finds

The average systemic sclerosis (SSc) patient burdened with pulmonary arterial hypertension (PAH) lives only four years after diagnosis, finds an Australian study,  “Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension,” that appeared in the journal Arthritis Research & Therapy.

PAH is a serious and potentially fatal condition affecting 8 to 15 percent of SSc patients. The past decade has seen an increase of therapeutic options for PAH, mostly using advanced pulmonary vasodilator therapies alone or combined with other treatments. These PAH therapies include endothelin receptor antagonists (ERA), phosphodiesterase-5-inhibitors (PDE5 and prostacyclin analogues) and their combinations (monotherapy or combination therapy).

Yet despite an improvement in survival thanks to these therapies, the outcomes for PAH patients with SSc still remains lower than for other PAH patients.

In the study, researchers aimed to “determine survival, predictors of mortality and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH.” They assessed SSc patients diagnosed with Group 1 PAH, according to the guidelines of the World Health Organization (WHO).

In total, the study analyzed 132 SSc-PAH patients from the Australian Scleroderma Cohort Study, which comprises 13 centers and is a prospective multi-center study of risk and prognostic factors for cardiopulmonary outcomes in SSc. During the study, which last from 2009 to 2015, 60 patients — or 45.5 percent of the total — died. Median survival time from PAH diagnosis was four years, though some lived only 2.2 years and others as long as 6.2 years.

Patients were followed from enrollment, for an average 3.8 years; 87.8 percent were still alive after one year, 78.3 percent after two years and 61.7 percent after three years. When comparing combination PAH therapy to monotherapy, the combo therapies showed a survival advantage. So did anticoagulation therapy versus no anticoagulation.

According to the team, “combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation.” However, anticoagulation in PAH remains controversial, with researchers noting that “further research is required to assess the role of anticoagulation in PAH specifically associated with SSc.”

The study identified older age at PAH diagnosis, mild co-existent interstitial lung disease, worse WHO functional class, higher mean pulmonary arterial pressure at PAH diagnosis and digital ulcers as independent predictors of mortality.

Overall, the team concluded, “despite advanced therapy, the median survival in SSc-PAH is only four years. In our study, the addition of anticoagulation to standard combination therapy was associated with a significant survival advantage.”