Abstract
Pulmonary arterial hypertension (PAH) develops in 7–12% of patients with systemic sclerosis (SSc) and is associated with a 3 year survival of 52%. Early detection by screening is therefore recommended for all patients with SSc. Historically, screening has been performed using echocardiography and measurement of gas transfer. More recently the DETECT protocol, using a combination of biomarkers (including N-terminal pro-brain natriuretic peptide) and clinical parameters, has been developed. The optimal method of screening for PAH with high sensitivity and specificity is, however, not clear. Protein expression differences between different SSc disease phenotypes have been reported, and include alterations in concentration of NT-proBNP, endoglin, soluble vascular endothelial growth factor receptor 1, placenta growth factor, growth differentiation factor-15, vascular endothelial growth factor alpha, resistin-like molecule beta, and soluble thrombomodulin. This review summarizes the current knowledge of these protein changes in patients with SSc and PAH.
Highlights
Pulmonary arterial hypertension (PAH) is a rare disease characterized by a progressive vasculopathy of the small pulmonary arteries leading to increased pulmonary vascular resistance and right ventricular afterload
We included studies identifying a cohort of patients diagnosed with systemic sclerosis with PAH with comparator groups including healthy volunteers (HV), systemic sclerosis without pulmonary hypertension (SSc-no PAH) and/or idiopathic PAH
A biomarker has been defined by the NIH as “a characteristic that is objectively measured and evaluated as an indicator of normal biological processes, pathogenic processes, or pharmacologic responses to a therapeutic intervention.”
Summary
Pulmonary arterial hypertension (PAH) is a rare disease characterized by a progressive vasculopathy of the small pulmonary arteries leading to increased pulmonary vascular resistance and right ventricular afterload. PAH may be idiopathic, heritable, related to various drugs and toxins or may be associated with a number of medical conditions including congenital systemic to pulmonary shunts, portal hypertension, HIV and connective tissue disease, most notably systemic sclerosis (SSc). These forms of PAH have similarities in underlying pathophysiology and treatment regime, it is increasingly recognized that there are differences in pathobiology, response to treatment and prognosis. These proteins may reflect the underlying pulmonary vascular disease as well as the response of the right ventricle to the increased afterload. This paper reviews the current published evidence base for altered protein biomarker concentrations in SSc-PAH
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