FRI0237 COMPARISON OF DIFFERENT PULMONARY HYPERTENSION SCREENING ALGORITHMS IN PATIENTS WITH SYSTEMIC SCLEROSIS
Authors: M. Erdogan, B. Kilickiran Avci, C. Ebren, Y. Ersoy, Z. Ongen, G. Ongen, V. Hamuryudan, G. Hatemi
Journal: Annals of the Rheumatic Diseases (2020)

Background: Pulmonary hypertension (PH) is an important cause of morbidity and mortality in patients with systemic sclerosis (SSc). Different screening algorithms have been proposed for identifying patients who have a high probability of PH and require right heart catheterization (RHC), which is the gold standard for diagnosing PH. Objectives: To compare the performance of PH screening algorithms in our patients with SSc. Methods: Sixty-nine consecutive pts fulfilling ACR/EULAR 2013 SSc criteria have been screened for PH until now, using the 2015 ESC/ERS, DETECT and ASIG algorithms. Pulmonary function tests (PFT), diffusing capacity of the lung for carbon monoxide (DLCO), trans-thoracic echocardiography, serum NT-proBNP and uric acid assay and high-resolution computed tomography (HRCT) were performed as needed. Patients with known PH, severe interstitial lung disease and severe left ventricular dysfunction (LVD) were not included. RHC was performed in all patients with positive screening according to any one of the screening algorithms. Pts with PH were classified according to the updated PH classification criteria. Sensitivity and specificity of the 3 screening algorithms were evaluated according to the established cut-off value of 25 mmHg for mean systolic pulmonary artery pressure and for the recently proposed cut-off value of 20 mmHg. Results: Among the 69 SSc pts, 27 were excluded due to ILD(n=6), LVD(n=6), already diagnosed PH(n=4) no measurable TRV(n=5), lung cancer (n=2), pulmonary embolism (n=1) and nephrotic syndrome (n=1). Among the remaining 42 patients, 17 required RHC according to at least one of the screening algorithms (Table 1). Number of patients who had suspected pulmonary hypertension and required RHC according to ESC/ERS 2015, DETECT and ASIG were 7 (%17), 13 (%31), and 12 (%29) respectively (Figure 1). Among the 17 pts. who had RHC, PH was present in 3 pts according to the 25-mmHg cut-off (Group 2 in 2, Group 3 in 1) and in 9 pts according to the 20-mmHg cut-off (Group 1 in 5, Group 2 in 3, Group 3 in 1). The sensitivity and specificities were presented in Table 2. ASIG and DETECT had better sensitivity for 25-mmHg cut-off and was better with ASIG for 20 mmHg cut-off. The specificity was better with ESC/ERS for both cut-off values. Conclusion: The ASIG algorithm has a better sensitivity and ESC/ERS algorithm has a better specificity for detecting PH in patients with SSc. A limitation of this study was that RHC was not performed in patients who did not fulfill criteria according to any of the screening algorithms. The sensitivities may be lower than what we propose if there are patients with PH who are asymptomatic and not captured with any of the algorithms. Disclosure of Interests: Mustafa Erdogan: None declared, Burcak Kilickiran Avci: None declared, Cansu Ebren: None declared, Yagmur Ersoy: None declared, Zeki Ongen: None declared, Gul Ongen: None declared, Vedat Hamuryudan Speakers bureau: Pfizer, AbbVie, Amgen, MSD, Novartis, UCB, Gulen Hatemi Grant/research support from: BMS, Celgene Corporation, Silk Road Therapeutics – grant/research support, Consultant of: Bayer, Eli Lilly – consultant, Speakers bureau: AbbVie, Mustafa Nevzat, Novartis, UCB – speaker