Screening patients with scleroderma for pulmonary arterial hypertension and implications for other at-risk populations.

ABSTRACT: Pulmonary arterial hypertension (PAH) is a progressive vasculopathy that is advanced by the time symptoms develop. As symptoms are nonspecific and the condition uncommon, continued progression toward end-stage disease occurs for an average of 2 years between symptom onset and diagnosis. There is need for earlier diagnosis and treatment, as most patients are severely symptomatic when diagnosed and their mortality is high despite therapy. Screening can help; however, it is not straightforward due to the diversity of patient profiles and lack of sufficiently accurate tools. Echocardiography, currently the best available screening tool, lacks both sensitivity and specificity. The low prevalence of PAH renders many screening tools unfit for purpose. However, this may be overcome, in some instan

SUBMITTER: Schwaiger JP 

PROVIDER: S-EPMC9639190 | biostudies-literature | 2013 Dec

REPOSITORIES: biostudies-literature