Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension

Abstract

N-terminal pro-brain natriuretic peptide (NT-proBNP) is a marker of neurohormonal activation that is useful in the diagnosis and prognosis of various forms of pulmonary arterial hypertension (PAH). We sought to characterise and compare NT-proBNP in a cohort of PAH related to systemic sclerosis (PAH-SSc) and idiopathic PAH (IPAH) patients.NT-proBNP levels, collected from PAH-SSc and IPAH patients followed prospectively, were compared and correlated with haemodynamic variables. Cox proportional hazard models were created to assess the predictive value of NT-proBNP.98 patients (55 PAH-SSc, 43 IPAH) were included. Haemodynamics were similar, except for lower mean pulmonary arterial pressure in PAH-SSc. NT-proBNP levels were significantly higher in PAH-SSc (3,419¡3,784 versus 1,393¡1,633 pg?mL -1 ; p,0.01) and were more closely related to haemodynamics in PAH-SSc than IPAH. 28 patients died. NT-proBNP predicted survival (hazard ratio (HR) 3.18; p,0.01) in the overall cohort; however, when stratified by group, predicted survival only in PAH-SSc (HR 3.07, p,0.01 versus 2.02, p50.29 in IPAH). This is the first description showing NT-proBNP levels are 1) significantly higher in PAH-SSc than IPAH despite less severe haemodynamic perturbations, and 2) stronger predictors of survival in PAH-SSc, suggesting that neurohormonal regulation may differ between PAH-SSc and IPAH. Future studies to define pertinent mechanisms are warranted.

Increased Expression of Growth Differentiation Factor-15 in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

“…We did not exclude subjects with interstitial lung disease in this study, which could have been a potential confounder. Finally, Mathai et al 25 recently reported that levels of NT-proBNP predicted survival in subjects with SSc-PAH, but not in those with IPAH. We did not find the same predictive strength for NT-proBNP in our study, possibly because of the larger number of subjects in the Mathai study.…”

Serum Endostatin Is a Genetically Determined Predictor of Survival in Pulmonary Arterial Hypertension

“…We evaluated the relationship between serum ES and clinical markers of exercise performance and function, specifically 6 MWD and NYHA-FC, and serum chemistries previously linked to outcomes in PAH, notably NT-proBNP (18)(19)(20), creatinine (21), and serum sodium (22) ( Table 2).…”

Plasma N-Terminal Pro-Brain Natriuretic Peptide Level was Lower in COPD Patients with Pulmonary Artery Hypertension

“…In 2006, Williams MH et al found that plasma NT-proBNP level was significantly higher in PAH patients associated with systemic sclerosis (PAH-SSc) compared with the SSc patients without PAH [17]. Recently, Mathai SC et al also found that plasma NT-proBNP level was significantly higher in PAH-SSc compared with idiopathic PAH (IPAH) patients and was a stronger predictor of survival in PAH-SSc than IPAH [18]. The results of Mathai's group suggested that the expression of NT-proBNP might be different in the different kinds of PAH.…”