title: 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 x 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, p = 0.29 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.
authors: Division of Pulmonary and Critical Care Medicine, Dept of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
authors: S C
authors: SC
authors: Mathai
authors: M
authors: M
authors: Bueso
authors: L K
authors: LK
authors: Hummers
authors: D
authors: D
authors: Boyce
authors: N
authors: N
authors: Lechtzin
authors: J
authors: J
authors: Le Pavec
authors: A
authors: A
authors: Campo
authors: H C
authors: HC
authors: Champion
authors: T
authors: T
authors: Housten
authors: P R
authors: PR
authors: Forfia
authors: A L
authors: AL
authors: Zaiman
authors: F M
authors: FM
authors: Wigley
authors: R E
authors: RE
authors: Girgis
authors: P M
authors: PM
authors: Hassoun
mesh_terms: D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D010446:Peptide Fragments / Q000097:blood*; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain