ABS0291 CLINICAL AND HEMODYNAMICS OUTCOMES PATIENTS WITH PULMONARY HYPERTENSION AND SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES

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Background: Pulmonary hypertension (PH) has big impact on patients with systemic autoimmune rheumatic diseases (SARD). PH haemodynamics (HD) criteria have been modified. However, the impact and evolution of HD parameters in PH-SARD subsets has not been studied in deep. Objectives: 1To describe the clinical and HD characteristics in a PH-SARD cohort. 2To determine factors that influence the outcomes of patients with PH-SARD. Methods: Retrospective study conducted at Spanish referral center on SARD. We recruited patients with PH-SARD diagnosis confirmed by right heart catheterization. The HD criteria used were according to the World Pulmonary Hypertension Association. Subset of SARD and subset of PH, demographic, clinical, PH screening tools and therapeutic data were recorded. HD parameters at diagnosis and during follow-up were also analyzed. HD was repeated in patients according to clinical criteria. Results: Thirty-four patients with PH-SARD were identified from June 2006 to November 2024 and a total of 55 HD studies have been done during the study period. Most of patients were women (29/34. The most frequent SARD was scleroderma (67.6%). 6/34 patients had overlapping syndrome (OS) and RA-SSc was present in 4 out 6 OS-PH patients. The PH subsets at diagnosis were PH group I (PAH:16/34), followed by group I+II (11/34), group II (PH-LHD: 6/34) and one pt group III (HP-ILD). 73.5% had ≥ 1 cardiovascular risk factor, 38.2% ILD, and 35.3% left heart disease prior to PH. Only two cases changed PH subset during the follow-up. A Half of patients received immunosuppressants previous PH diagnosis and 64.7%, 11.8% and 5.9% received calcium channel blockers (CCB), endothelin receptor antagonists (ERA) or phosphodiesterase-5 inhibitors before PH was detected. 6 patients died during the study period. The characteristics of the sample are summarized in Table 1. The HD control study was performed at mean 4.6±3.4 years during the follow-up with no differences between parameters. However, patients with OS-PH showed higher mPAP at PH diagnosis (p<0.02) and higher cardiac output (CO) at follow-up (p0.02). Is note to worth that all deceased patients did not take immunosuppressive treatment previous to PH detection (p<0.02). The deceased patients also were older (p<0.03) and had lower PAWP values (p<0.03). Patients who received CCB took longer to present PH (p<0.02) but the CO at diagnosis was worse (p<0.04). Patients with previous treatment with ERA showed longer time free of PH (p<0.05). No differences were identified between the deceased patients and the PH subgroup nor kind of SARD. Table 1 . Conclusion: Patients with OS-HP presented different HD profile at diagnosis and during follow-up compared to others PH-SARD. There were no deaths in patients with SARD and previous immunosuppressive treatment. Patients with ERA treatment before PH diagnosis showed longer time without PH during the follow-up. More specific studies are needed to know the behavior of PH in each SARD. REFERENCES: [1] Humbert M, Kovacs G, Hoeper MM, et al. ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43(38):3618-3731. Acknowledgements: NIL . Disclosure of Interests: Ivan Castellvi Boehringher-Ingelheim, GSK, Novartis, Boehringher-Ingelheim, GSK, Johnson & Johnson, Boehringer-Ingelheim, Novartis, Sanofi, Johnson & Johnson, Kern, Andrea Hernández: None declared , Helena Codes Abbvie, Jose Luis Tandaipan Johnson & Johnson, Ivette Casafont-Solé GSK, Johnson & Johnson, Joel Francesqui Bial, Boehringher-Ingelheim, Mireia Padilla López: None declared , Julia Bernardez Vifor, Patricia Moya: None declared , Berta Paula Magallares GSK, Astra Zeneca, Hye Sang Park Lilly, Pfizer, Alfa Sigma, Janssen, Novartis, MSD, Boehringer-Ingelheim and Amgen, Luís Sainz Comas: None declared , Asier García-Alija Amgen, Albert Casals Urquiza: None declared , Guillem Verdaguer: None declared , Susana P. Fernandez-Sanchez GSK, Astra Zeneca, Mari-Angels Sendra: None declared , Sandra Ros: None declared , Cesar Díaz-Torné: None declared , Ana Laiz ABBVIE, JANSSEN, UCB, NOVARTIS, UCB, Marta Camprecios: None declared , Diego Castillo: None declared , Hèctor Corominas: 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 (http://creativecommons.org/licenses/by-nc-nd/4.0/). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.

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