Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody.
One in six patients with systemic sclerosis will develop pulmonary arterial hypertension (PAH). Screening with echocardiography and possibly pulmonary function testing (to determine the diffusing capacity of carbon monoxide) is recommended to detect PAH at a less severe stage. However, real-world screening programs have problems. Registries where echocardiograms are to be performed annually should have the best-case scenario of nearly perfect screening and referral for right heart catheterization of those highly suspect for PAH. However, registries demonstrate care gaps where patients are not referred for appropriate confirmatory testing when PAH is suspected.