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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 Forty-eight consecutive patients, fulfilling ACR/EULAR 2013 SSC criteria, were screened for PH 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, 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 were not included. RHC was performed in all patients with positive screening according to any one of the screening algorithms. Patients 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 48 SSc patients, 15 were excluded due to already diagnosed PH (n=4), left ventricular dysfunction (n=4), no measurable tricuspid regurgitation velocity (TRV) (n=5) and coexisting lung cancer (n=2). Among the remaining 34 patients, 16 required RHC according to at least one of the screening algorithms. Demographic and clinical features of remaining 34 patients were summarized in Table 1. Number of patients who had suspected pulmonary hypertension and required RHC according to ESC/ERS 2015, DETECT and ASIG were 8 (%25), 9 (%27), and 13 (%41) respectively (Figure 1). Among the 14 who had RHC, PH was present in 3 patients according to the 25-mmHg cut-off (Group1 in 1, Group 2 in 1, Group 3 in 1) and in 8 patients according to the 20-mmHg cut-off (Group 1 in 5, Group 2 in 2, Group 3 in 1). The sensitivity and specificity of each algorithm is presented in Table 2. Sensitivity was similar at 100% for the 3 algorithms, but the ESC/ERS algorithm had better specificity, when PH was diagnosed with the 25-mmHg cut-off. For the 20-mmHg cut-off, both the sensitivity and the specificity were better with the ESC/ERS algorithm. Conclusion The ESC/ERS algorithm seems to have a better performance 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, Yagmur Ersoy: None declared, Cansu Ebren: None declared, Zeki Ongen: None declared, Gul Ongen: None declared, Vedat Hamuryudan Consultant for: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB, Speakers bureau: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB, Gulen Hatemi Consultant for: Abbvie, Amgen, BMS, Janssen, MSD, Pfizer, UCB, Speakers bureau: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB |
