Abstract

Abstract Background/Aims Pulmonary arterial hypertension (PAH) affects approximately 10% of patients with SSc and is a major cause of morbidity and mortality. PAH is often asymptomatic, and therefore, proactive screening is crucial for early detection and improved survival, including through early treatment intervention. Although there are various proposed international guidelines for screening, there is currently no dedicated UK-based guidance. Our aim is to explore current UK screening practices for SSc-PAH patients, including identifying barriers and potential solutions. Methods A survey of 31 questions was developed and included six sections: clinician demographics, the importance of screening, screening practices, barriers to screening, treatment, and patient education. The survey was disseminated among rheumatologists working in the UK. Results Forty-four rheumatologists working in the UK participated in the study, and the majority completed all the questions. Around one-third (37.0%) worked in specialised SSc units (university or general hospitals [54.5% and 45.4%, respectively]). The majority recognised that SSc-PAH is a major cause of morbidity and mortality. Over half (60.0%) reported using the DETECT algorithm to screen for SSc-PAH, although other algorithms were also sometimes used. All of the respondents utilised transthoracic echocardiogram, and almost all (95.0%) performed pulmonary function tests for screening purposes. Various challenges and barriers were identified relating to SSc-PAH screening (Figure 1A), with the difficulty in interpreting results from other hospitals and extended wait times for diagnostic tests being the most reported (76.0% and 74.0%, respectively). Most respondents agreed that access to key investigations (87.0%), ongoing clinician education (82.0%), multidisciplinary meetings (79.5%) and a better understanding of proposed screening algorithms (79.5%) could be potential solutions (Figure 1B). Conclusion Rheumatologists recognise that SSc-PAH screening is crucial to improve outcomes including survival; however, variable practices exist. There are various barriers to SSc-PAH screening and we have identified a number of practical potential solutions. Disclosure M.P. Álvarez Hernández: None. Y. Allanore: Honoraria; Speaking fees from Janssen outside the submitted work. I. Andrade: Other; Honoraria (speaker) fees, scientific board member for EchoRight™and advisory board participant for ‘Pulmonary hypertension in the primary care setting’ for Janssen, outside the submitted work. M.H. Buch: Other; Speaker fees from Boehringer Ingelheim, outside the submitted work. G. Coghlan: Other; Consultancy and working groups fees from Janssen and MSD; honoraria from Inari and Janssen; grants from Janssen. F. Del Galdo: Other; Consulting fees and research support from Janssen, outside the submitted work. C. Denton: Honoraria; Consulting fees and research support from Janssen, outside the submitted work. D. Khanna: Honoraria; Consultant: Amgen, Abbvie, BI, AZ, Merck, Horizon, Prometheus, Janssen, GSK. Grants- Pfizer, BI, Horizon. D.G. Kiely: Honoraria; Participation in advisory boards, steering committees, speaker fees and support to attend educational meetings from Acceleron, Gossamer, GSK, Janssen, Ferrer, MSD and United Therapeutics. Other; grant funding from GSK and Janssen. His work is supported by a grant from the NIHR Biomedical research Centre Sheffield. J. Pauling: Honoraria; Consultancy work for AstraZeneca, Boehringer Ingelheim, IsomAb, Janssen, Permeatus and Sojournix; speaker honoraria and educational support from Janssen. S. Ramjug: Consultancies; Consulting fees from Janssen, outside the submitted work. M. Hughes: Other; Speaking fees from Actelion, Eli Lilly, Janssen and Pfizer, outside the submitted work; research funding from Janssen; member of a data and safety monitoring board for Certa Therapeutics.

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