SAT0308 SCREENING TOOLS FOR PULMONARY ARTERIAL HYPERTENSION (PAH) IN SYSTEMIC SCLEROSIS (SSC): A SYSTEMATIC LITERATURE REVIEW (SLR).
Authors: C. Bruni, G. De Luca, M. G. Lazzaroni, E. Zanatta, G. Lepri, M. Matucci-Cerinic
Journal: Annals of the Rheumatic Diseases (2020)

Background: in SSc, PAH has a high morbidity and mortality burden. Therefore, screening and early detection are pivotal to achieve an early diagnosis of PAH. Objectives: to search the literature for all screening modalities for SSc-PAH in reference to right heart catheterization as diagnostic gold standard. Methods: papers from 2 previously published SLRs [22 from Glaude et al (1) – from inception to 19/06/2012 - and 22 from Young et al 2018 – from 20/06/2012 to 02/10/2017] were included. The articles’ database was integrated with a systematic search on Pubmed, EMBASE, Web of Science for papers published from 03/10/2017 to 31/12/2018. A total of 199 papers were reviewed and 32 were finally extracted. Bias risk was assessed through QUADAS2 tool. Results: 167 papers were excluded from data extraction mainly for PAH screening non as main focus or for non-including SSc patients. The 32 papers extracted presented a low bias risk according to QUADAS2. Screening methods reported were: Echocardiographic parameters in 31/32 studies, in particular systolic pulmonary arterial pressure (sPAP) in 22 papers; 40 mmHg was the most frequently used cut-off (in 12/22 papers); sPAP was part of a composite algorithm in 9/22 papers. Among others, tricuspid regurgitation velocity (TRV) was used in 6/31 (as part of composite 5/6) and right atrial pressure (RAP) in 3/31 papers. Pulmonary function tests parameters in 22/32 papers, with % predicted Lung diffusion for carbon oxyde (DLco) in 21 papers, with a 50% cut-off in 11/21 and as part of composite algorithm in 13/21 studies. Moreover, walked distance at six minutes walking test was a screening parameter in 3/32 papers. Serum biomarkers in 12/32 papers, with anti-centromere antibodies (6/12), NT-proBNP (6/12) and uric acid (5/12) being the most frequently reported. Clinical parameters in 15/32 papers, with unexplained dyspnoea in 9/15 and telangiectasias in 5/15 papers. Composite algorithms were used in 18/32 manuscripts: among them, DETECT (5/18), ESC/ERS 2009 (4/18) or 2015 (3/18) guidelines, ASIG (2/18) e ITINER-air (1/18). In different cohorts, DETECT and ASIG showed higher sensitivity and negative predictive value than ESC/ERS 2009. Conclusion: in the literature, the screening of SSc-PAH is largely investigated by echocardiographic parameters. In particular, sPAP and TRV, both as single items or part of a composite algorithm, including also serum biomarkers, clinical and functional parameters, are the most frequent parameters evaluated. (supported by an Actelion Pharmaceuticals unrestricted research grant) References: [1]Gladue H, et al. Screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension: a systematic review. Semin Arthritis Rheum. 2014 Feb;43(4):536-41. [2]Young A, et al. Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension. Semin Arthritis Rheum. 2019 Jun;48(6):1059-1067. Acknowledgments: (supported by an Actelion Pharmaceuticals unrestricted research grant) Disclosure of Interests: Cosimo Bruni Speakers bureau: Actelion, Eli Lilly, Giacomo De Luca Speakers bureau: SOBI, Novartis, Celgene, Pfizer, MSD, Maria Grazia Lazzaroni: None declared, Elisabetta Zanatta: None declared, Gemma Lepri: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim