Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension

Abstract

Raised N-TproBNP levels are directly related to the severity of PAH. In screening programs, SSc patients with an N-TproBNP in excess of 395 pg/mL have a very high probability of having pulmonary hypertension. Baseline and serial changes in N-TproBNP levels are highly predictive of survival. A 10-fold increase in N-TproBNP level on therapy is associated with a greater than three-fold increase in mortality, and may indicate therapeutic failure.

Biomarkers in pulmonary hypertension

“…Here, a high negative predictive value of 96% was found but the positive predictive value was lower at 69.2%, suggesting that NT-proBNP may be of more use in excluding rather than diagnosing the condition. However, a more recent study in a larger number of scleroderma patients, in whom right heart catheterisation was performed, found a higher positive predictive value of 95%, and lower negative predictive value of 56.5% [33]. In a screening study of 39 patients with pulmonary fibrosis of different aetiologies, BNP level predicted moderate-severe PH with 100% sensitivity and 89% sensitivity [15].…”

“…In systemic sclerosis, NT-proBNP is elevated in patients with PAH and correlates with pulmonary haemodynamics [32,33]. Levels are elevated in patients with sickle cell disease compared with healthy, black controls, and are higher still in those patients with PAH [34].…”

“…One such high-risk group is patients with limited scleroderma, in whom the prevalence of PAH is estimated to be o12% [26]. NT-proBNP has been evaluated as a marker of early PAH in patients with systemic sclerosis [32,33]. NT-proBNP has also been used to screen for PH in patients with sickle cell disease [34].…”

“…However, in some conditions there may be coexistent features of the disease that may influence these measures and thus they may not truly represent a patient's clinical state, e.g. musculoskeletal involvement in systemic sclerosis [33]. Biomarkers can potentially offer additional information to the clinician for monitoring the efficacy of treatment and the patient's clinical course.…”

Biomarkers in Systemic Sclerosis

“…Recent interest has also been focused on the measurement of plasma levels of NT-pro-BNP. Although the plasma levels of this peptide reflect myocardial responses to various stimuli, such as mechanical stretch or hypoxia and are not specific for PAH, plasma NT-pro-BNP determinations may predict prospectively the development of clinical PAH, and may also be indicative of survival and represent an accurate surrogate marker to follow the response and evaluate the effects of therapeutic agents for SSc-related PAH [64][65][66][67][68].…”