Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) who were administered combination sequential therapy had improved survival rates compared with patients receiving upfront combination or monotherapy, according to study results published in The Journal of Rheumatology.

Researchers conducted a retrospective cohort study to examine data from patients with SSc-PAH in the Spanish Scleroderma Registry (RESCLE) database. The patient population was split into 3 groups: monotherapy (endothelin receptor antagonists [ERA] or phosphodiesterase-5 [PDE-5] inhibitors), sequential combination therapy, or upfront combination therapy. Sequential combination therapy is defined by ≥12 weeks between initiation of the first and second drug, while upfront combination therapy is defined by <12 weeks between the first and second drug. The primary outcome was death from any cause at 1, 3, and 5 years from diagnosis.

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Of the 1817 patients with SSc in the RESCLE database, 76 had SSc-related PAH and were being treated with either ERA, PDE-5 inhibitors, or both. Of these 76 participants, 34 were receiving monotherapy with ERA or PDE-5 inhibitors, 25 were given the sequential combination, and 17 were given upfront combination therapy. After a multivariate analysis, both prescribed treatment regimens (P =.017) and functional status at baseline were found to be independent predictors of long-term mortality.

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Long-term survival rates from diagnosis of SSc-related PAH at 1, 3, and 5 years were 78.0%, 40.7%, and 31.6%, respectively, in the monotherapy group; 95.8%, 81.5%, and 56.5%, respectively, in the sequential combination group; and 94.1%, 51.8%, and 34.5%, respectively, in the upfront combination group.

“[O]ur study results suggest that combination therapy is superior to monotherapy in terms of survival,” the researchers wrote. “[T]he most important strength of the present study is the fact that the investigation only focused on the specific group of SSc-related PAH patients, and evaluated long-term mortality as a single end point instead of a combined end point of morbidity and mortality.”


Pestaña-Fernández M, Rubio-Rivas M, Vilella CT, et al; on behalf of RESCLE Investigators, Autoimmune Diseases Study Group (GEAS). Long-term efficacy and safety of monotherapy versus combination therapy in systemic sclerosis-associated pulmonary arterial hypertension: a retrospective cohort study from the Nationwide Spanish Scleroderma Registry (RESCLE) [published February 15, 2019]. J Rheumatol. doi:10.3899/jrheum.180595