title: 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 instances, by using enrichment tools to preselect screening populations. The majority of data are available for systemic sclerosis. A recent study has demonstrated how lung function can be used to enrich PAH prevalence in a systemic sclerosis population. A screening bundle then selects patients for right heart catheterisation with improved rates of sensitivity compared to current guidelines.
authors.affiliation: Dept of Cardiology, Royal Free Hospital, London, UK. 2 University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Johannes P
authors.initials: JP
authors.lastname: Schwaiger
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.forename: J
authors.initials: J
authors.lastname: Gerry Coghlan
mesh_terms: D000465:Algorithms; D015415:Biomarkers / Q000097:blood; D019937:Diagnostic Techniques and Procedures / Q000592:standards; D018450:Disease Progression; D042241:Early Diagnosis; D004452:Echocardiography; D005080:Exercise Test; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008168:Lung / Q000503:physiopathology; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D011379:Prognosis; D012129:Respiratory Function Tests; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D013997:Time Factors
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain