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

• Published in: Clinical rheumatology Vol. 43; no. 6; pp. 1919 - 1925
• Main Authors: Sarı, Alper, Satış, Hasan, Ayan, Gizem, Küçükşahin, Orhan, Kalyoncu, Umut, Fidancı, Ali Aykut, Ayvalı, Mustafa Okan, Ata, Naim, Ülgü, Mustafa Mahir, Birinci, Şuayip, Akdoğan, Ali
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01-06-2024; Springer Nature B.V
• Subjects: Aged; Antihypertensive Agents - therapeutic use; Diagnosis; Female; Humans; Hypertension; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - mortality; Immunosuppressive agents; Immunosuppressive Agents - therapeutic use; Kaplan-Meier Estimate; Lung diseases; Male; Medical prognosis; Medicine; Medicine & Public Health; Middle Aged; Original Article; Patients; Pulmonary Arterial Hypertension - drug therapy; Pulmonary Arterial Hypertension - mortality; Pulmonary hypertension; Retrospective Studies; Rheumatology; Scleroderma; Scleroderma, Systemic - complications; Scleroderma, Systemic - mortality; Survival; Survival Rate; Systemic sclerosis; Turkey - epidemiology; Turkey; Pulmonary arterial hypertension; Mortality; Systemic sclerosis; Nationwide
• ISSN: 0770-3198; 1434-9949; 1434-9949
• DOI: 10.1007/s10067-024-06961-0

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.