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. 2024 Jun;43(6):1919-1925.
doi: 10.1007/s10067-024-06961-0. Epub 2024 Apr 27.

Survival in systemic sclerosis associated pulmonary arterial hypertension in the current treatment era-results from a nationwide study

Alper Sarı  1 , Hasan Satış  2 , Gizem Ayan  3 , Orhan Küçükşahin  4 , Umut Kalyoncu  3 , Ali Aykut Fidancı  5 , Mustafa Okan Ayvalı  5 , Naim Ata  5 , Mustafa Mahir Ülgü  5 , Şuayip Birinci  6 , Ali Akdoğan  3
Affiliations

Survival in systemic sclerosis associated pulmonary arterial hypertension in the current treatment era-results from a nationwide study

Alper Sarı et al. Clin Rheumatol. 2024 Jun.

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). This nationwide study aims to describe real world treatment characteristics and assess survival rates of patients with SSc-PAH.

Methods: In this retrospective cohort study, patients with SSc-PAH were identified from Turkish Ministry of Health National Electronic Database (from January 2016 to September 2022), using ICD-10 codes. Data on demographics, treatment characteristics, and death was collected. Kaplan-Meier curves were used to calculate cumulative probabilities of survival at 1, 3, and 5 years.

Results: Five hundred forty-seven patients (90.7% female) with SSc-PAH were identified. Median age at PAH diagnosis was 59.9 (50.0-67.4) years. During a median follow-up duration of 3.2 (1.5-4.8) years, 199 (36.4%) deaths occurred. Estimated survival rates at 1, 3, and 5 years were 90.2%, 73.2%, and 56.6%, respectively. Survival was similar among patients with and without interstitial lung disease (p = 0.20). Patients who used immunosuppressives had better survival than those who did not (p < 0.001). No difference was observed in survival rates according to initial PAH-specific treatment regimen (monotherapy or combination) (p = 0.49).

Conclusion: Compared to most of historical cohorts, higher survival rates for SSc-PAH were observed in this study. Early diagnosis of PAH may have contributed to these findings. The impact of immunosuppressive therapy on prognosis of SSc-PAH needs to be further investigated in prospective studies. Key Points • Early diagnosis is pivotal for better outcomes in SSc-PAH. • Implementation of PAH treatment guidelines in routine clinical practice is still poor and should be improved. • Effect of immunosuppressive therapies on disease course has to be defined in SSc-PAH.

Keywords: Mortality; Nationwide; Pulmonary arterial hypertension; Systemic sclerosis.

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