{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T05:25:17Z","timestamp":1787030717192,"version":"build-2736575974"},"reference-count":42,"publisher":"The Journal of Rheumatology","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:sec>\n                    <jats:title>Objective.<\/jats:title>\n                    <jats:p>Monotherapy is an option as first-line therapy for pulmonary arterial hypertension (PAH). However, combination therapy is a beneficial alternative. Our objective was to evaluate the efficacy of monotherapy versus combination therapy in patients with systemic sclerosis (SSc)\u2013associated PAH.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods.<\/jats:title>\n                    <jats:p>All patients with SSc-associated PAH from the Spanish Scleroderma Registry (RESCLE) were reviewed. Patients were split into 3 groups: monotherapy versus sequential combination versus upfront combination therapy. The primary endpoint was death from any cause at 1, 3, and 5 years from PAH diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results.<\/jats:title>\n                    <jats:p>Seventy-six patients (4.2%) out of 1817 had SSc-related PAH. Thirty-four patients (45%) were receiving monotherapy [endothelin receptor antagonist (n = 22; 29%) or phosphodiesterase-5 inhibitors (n = 12; 16%)], 25 (33%) sequential combination, and 17 (22%) upfront combination therapy. A lower forced vital capacity\/DLCO in the sequential combination group was reported (2.9 \u00b1 1.1 vs 1.8 \u00b1 0.4 vs 2.3 \u00b1 0.8; p = 0.085) and also a higher mean pulmonary arterial pressure in combination groups (37.2 \u00b1 8.7 mmHg vs 40.8 \u00b1 8.8 vs 46 \u00b1 15.9; p = 0.026) at baseline. Treatment regimen (p = 0.017) and functional class (p = 0.007) were found to be independent predictors of mortality. Sequential combination therapy was found to be an independent protective factor (HR 0.11, 95% CI 0.03\u20130.51; p = 0.004), while upfront combination therapy showed a trend (HR 0.68, 95% CI 0.23\u20131.97; p = 0.476). Survival from PAH diagnosis among monotherapy, sequential, and upfront combination groups was 78% versus 95.8% versus 94.1% at 1 year, 40.7% versus 81.5% versus 51.8% at 3 years, and 31.6% versus 56.5% versus 34.5% at 5 years (p = 0.007), respectively. Side effects were not significantly different among groups.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion.<\/jats:title>\n                    <jats:p>Combination sequential therapy improved survival in our cohort.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3899\/jrheum.180595","type":"journal-article","created":{"date-parts":[[2019,2,15]],"date-time":"2019-02-15T12:20:55Z","timestamp":1550233255000},"page":"89-98","source":"Crossref","is-referenced-by-count":17,"title":["Longterm Efficacy and Safety of Monotherapy versus Combination Therapy in Systemic Sclerosis\u2013associated Pulmonary Arterial Hypertension: A Retrospective RESCLE Registry 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