Background Systemic sclerosis (SSc) has a wide spectrum of cardiovascular manifestations, being pulmonary hypertension (PH) one of the most lethal complications. Echocardiography is a pivotal screening test for patients at risk for PH that allows prompt identification of those with high estimated pulmonary artery systolic pressure (PASP).
Objectives We conducted a retrospective analysis of patients with SSc followed in our Rheumatology department aiming to characterize those with estimated PASP≥35 mmHg.
Methods Clinical and laboratory features, including dyspnea according to New York Heart Association (NYHA) classes, N-terminal pro-Brain Natriuretic Peptide (NT-proBNP) serum levels, uricemia, anti-nuclear antibody (ANA) specificities, 6-minute walk test (6MWT), and right heart catheterization (RHC), if required, were assessed.
Results In total, 87 out of 103 SSc patients had available echocardiography results (females 93.1%; mean age 57.8 years; mean disease duration 10.3 years). Eight patients were classified as having NYHA class≥3. The median value of NTproBNP was 104 pg/mL (19 to 15902). Twenty one (24%) had estimated PASP≥35 mmHg (in 16 patients PASP could not be estimated), of whom16 performed RHC. PH (mPAP≥25 mmHg) was confirmed in 7 (44%) cases; 3 were classified as group 1, 1 as group 2, 2 as group 3 and 1 as group 5, according to the ERS/ECS 2013 classification. The group with elevated PASP included 11 patients with limited SSc, 7 with diffuse SSc, 1 sine scleroderma and 2 overlap syndromes. Comparison of patients with PASP≥35 mmHg with those with PASP<35 mmHg put in evidence the following differences (Table bellow).
Conclusions PASP is elevated in one quarter of SSc patients. This subgroup present more frequently higher NYHA classes, elevated NTproBNP levels and Scl70 positivity. About 44% of them had restrictive pulmonary disease. It is essential to closely monitor cardiorespiratory function in order to rule out cardiac involvement as early as possible.
Our results suggest that there is still need to increase physicians' awareness of systematic cardiovascular screening, since fewer patients than are nowadays recommended had a complete evaluation.
Acknowledgement Cardiology department Hospital Garcia de Orta
Disclosure of Interest None declared