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Background: Systemic sclerosis (SSc) is a rare systemic autoimmune disease associated with significant morbidity and mortality due to severe multi-organ involvement [1]. Pulmonary hypertension (PH) is a frequent and often life-threatening complication of SSc, with an estimated 1-year survival of about 80% and a 3-year survival close to 50% [2]. Pulmonary arterial hypertension (PAH) is an independent risk factor for mortality in SSc, and the severity of PAH predicts mortality in those with SSc-PAH [3]. Selexipag, a selective prostacyclin receptor agonist, has been approved since 2017 for the tratment of SSc-PAH, however only limited data on its use are available [4].
Objectives: This study aims to evaluate the long-term safety and clinical outcomes of Selexipag treatment in a real-life cohort of Italian SSc patients complicated by PAH.
Methods: Patients with diagnosis of SSc meeting the 2013 ACR/EULAR classification criteria [5], complicated by PAH diagnosed with right heart catheterism (RHC) [6], and treated with Selexipag, were retrospectively included. Demographic, clinical and laboratory data were analyzed with StataMP 18 software with appropriate statistical tests.
Results: Ten SSc-PAH patients (90% female), median (interquartile range (IQR)) age 71 (66-78) years, were treated with Selexipag for a median (IQR) of 26 (12-53) months (Table 1). The median (IQR) age of SSc diagnosis was 56 (51-65) years, while median (IQR) age of PAH diagnosis was 65 (63-72) years, with a median (IQR) SSc and PAH duration of 13 years (11-20 years) and 7 years (4-8 years), respectively. The majority of patients had limited cutaneous (lc) SSc involvement (60%), while 3 had sine-scleroderma (30%) and one (10%) diffuse cutaneous involvement. All patients had Raynaud phenomenon, active or late scleroderma pattern at capillaroscopy and ANA positive antibodies at variable titers, while anti-CENP antibodies were positive in 6 patients (60%). In our cohort median (IQR) modified Rodnan skin score (mRSS) was 2 (0-3), and most patients presented interstitial lung disease (ILD) (90%), gastrointestinal involvement (90%) and telangiectasias (90%). At diagnostic RHC, the median (IQR) of the mean pulmonary artery pressures was 31 (27-35) mmHg, median (IQR) pulmonary vascular resistance was 3.6 (2.3-5.2) Wood Units (WU), while median (IQR) pulmonary capillary wedge pressure (PCWP) was 11 (7-12) mmHg. In our cohort, the optimized and maximally tolerated Selexipag dose was median (IQR) 1200 (600-2200) microg/day. Most frequently prescribed DMARD was mycophenolate mofetil (40%), followed by hydroxychloroquine (30%) and colchicine (20%). Other treatments administered are summarized in Table 2. After a median (IQR) follow-up duration of 8 years (7-10 years), survival rate was 60%, with 4 exitus (two respiratory failure, one severe SARS-CoV-2 infection and one decompensated cirrhosis). In 5 SSc-PAH patients (50%) oxygen therapy was administered due to persistent dyspnea on exertion, with a median (IQR) NYHA functional class (FC) of 3 (2-3). No adverse events causing Selexipag treatment discontinuation were observed. All patients received appropriate therapeutic adjustments during follow-up and were under triple therapy for PAH at last follow-up visit.
Conclusion: Selexipag treatment represents a valuable therapeutic option for SSc-PAH patients, with a reassuring long-term safety profile and encouraging survival data.
REFERENCES: [1] Young A et al. J Clin Rheumatol . 2015;21(3):149-155.
[2] Lefèvre G et al. Arthritis & Rheumatism . 2013;65(9):2412-2423.
[3] Naranjo M et al. Diagnostics . 2021;11(5):911.
[4] Lemmers JM et al. Journal of Scleroderma and Related Disorders . 2020;5(3):NP7-NP11.
[5] van den Hoogen F et al. Ann Rheum Dis . 2013;72(11):1747-1755.
[6] Humbert M et al. European Heart Journal . 2022;43(38):3618-3731.
Demographic and clinical characteristics of SSc-PAH patients treated with Selexipag.
| Characteristics | SSc-PAH patients (n.10) |
|---|---|
| Female, n. (%) | 9 (90%) |
| Age, years (median, IQR 25-75) | 71 (66-78) |
| BMI (kg/m 2 ) (median, IQR 25-75) | 22 (17-25) |
| SSc disease duration, years (median, IQR 25-75) | 13 (11-20) |
| PAH duration, years (median, IQR 25-75) | 7 (4-8) |
| Autoimmune thyroid disease, n. (%) | 5 (50%) |
| Osteoporosis, n. (%) | 6 (60%) |
| Digital ulcers, n. (%) | 2 (20%) |
| Pitting scars, n. (%) | 5 (50%) |
| Acral osteolysis, n. (%) | 2 (20%) |
| NT-proBNP (pg/mL) (median, IQR25-75) | 600 (333-1330) |
Abbreviations: SSc, Systemic sclerosis; PAH, pulmonary arterial hypertension; IQR, interquartile range; BMI, body mass index; NT-proBNP, N-terminal pro–B-type natriuretic peptide.
Therapy administered in SSc-PAH patients treated with Selexipag.
| Therapy | SSc-PAH patients (n.10) |
|---|---|
| Antihypertensive drugs, n. (%) | 9 (90%) |
| Calcium antagonists, n. (%) | 2 (20%) |
| CCS therapy, n. (%) | 6 (60%) |
| CCS PDNeq dose (mg/day), (median IQR25-75) | 5 (5-5) |
| Sildenafil, n. (%) | 3 (30%) |
| Tadalafil, n. (%) | 6 (60%) |
| Macitentan, n. (%) | 8 (80%) |
| Ambrisentan, n. (%) | 2 (20%) |
| Prostanoids, n. (%) | 2 (20%) |
| Riociguat, n. (%) | 1 (10%) |
Abbreviations: SSc, Systemic sclerosis; PAH, pulmonary arterial hypertension; CCS, corticosteroids; PDNeq, prednisone equivalent dose; IQR, interquartile range.
Acknowledgements: NIL.
Disclosure of Interests: None declared.
© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (