NPPB and pulmonary arterial hypertension: Screening should include specific autoantibodies (anti-topoisomerase I (SCL-70), an-ti-centromere and anti-RNA polymerase III and antiphospholipid antibodies), pulmonary function tests, echocardiography, pro-terminal brain natriuretic peptide (NT-proBNP), capillaroscopy of nail folds and initial high-resolution CT scan to rule out ILD, and, in case of PAH, right heart catheterization to determine PA pressure [118,119,120,121,122,123,124,125,126,151].