N-Terminal Pro-Brain Natriuretic Peptide Is Disproportionately Elevated in Scleroderma Associated Pulmonary Arterial Hypertension Compared to Idiopathic Pulmonary Arterial Hypertension

Alexander Hannan¹, Raed Dweik², Kristin B. Highland³, Gustavo Heresi⁴, Adriano Tonelli⁵, William Messner⁶ and Soumya Chatterjee^1,7, ¹Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, ²Respiratory Institute, Cleveland Clinic, Cleveland, OH, ³Rheumatology.org, Cleveland Clinic, Cleveland, OH, ⁴Respiratory Institute - Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, ⁵Pulmonary Medicine - Respiratory Institute, Cleveland Clinic, Cleveland, OH, ⁶Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, ⁷Rheumatic and Immunologic Ds, Cleveland Clinic, Cleveland, OH

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: NT-proBNP, Pulmonary Involvement and scleroderma

Session Information

Date: Sunday, November 5, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: N-Terminal pro-brain natriuretic peptide (NT-proBNP) level tends to correlate with myocardial wall stress and/or myocardial damage (fibrosis). Hence, it has been promoted as a potential biomarker to support the diagnosis of pulmonary arterial hypertension (PAH) and help monitor its severity, progression, and response to therapy. A previous study has noted disproportionately elevated serum NT-proBNP levels among patients with scleroderma associated PAH (SSc-PAH) compared to similar patients with idiopathic PAH (iPAH).¹Our goal was to (1) verify the above results using a much larger cohort of SSc-PAH and iPAH patients, and (2) to identify possible explanations for this phenomenon.

Methods: A retrospective chart-review was conducted comparing demographic, laboratory, and hemodynamic [echocardiographic and right heart catheterization (RHC)] data from a total of 862 patients (686 with iPAH and 176 with SSc-PAH) enrolled in the Cleveland Clinic Pulmonary Hypertension Database. The diagnosis of PAH was confirmed by RHC, and the diagnosis of SSc was confirmed by a rheumatologist. NT-proBNP levels were measured at the time of the patient’s first RHC, which was also considered to be the time of PAH diagnosis. Mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) [data from RHC] and estimated right ventricular systolic pressure [data from transthoracic echocardiogram] were collected in both SSc-PAH and iPAH patients. A multivariate linear regression with a disease x log(NT-proBNP) interaction term was performed.

Results: In our cohort, at the time of first RHC, SSc-PAH patients were noted to have significantly higher NT-proBNP levels, yet much lower PVR and mPAP readings compared to similar iPAH patients. Also, there was a significant, positive correlation between NT-proBNP levels, mPAP, and PVR only in SSc-PAH patients, but not in iPAH patients.

Conclusion: One plausible explanation for higher NT-proBNP levels in SSc-PAH patients is intrinsic subclinical myocardial damage from SSc-induced fibrosis (which does not occur in early iPAH), in addition to right ventricular wall stress resulting from the occlusive pulmonary vasculopathy.

Reference: Mathai S et al. Eur Respir J 2010; 35: 95–104

Disclosure: A. Hannan, None; R. Dweik, None; K. B. Highland, None; G. Heresi, None; A. Tonelli, None; W. Messner, None; S. Chatterjee, None.

To cite this abstract in AMA style:

Hannan A, Dweik R, Highland KB, Heresi G, Tonelli A, Messner W, Chatterjee S. N-Terminal Pro-Brain Natriuretic Peptide Is Disproportionately Elevated in Scleroderma Associated Pulmonary Arterial Hypertension Compared to Idiopathic Pulmonary Arterial Hypertension [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/n-terminal-pro-brain-natriuretic-peptide-is-disproportionately-elevated-in-scleroderma-associated-pulmonary-arterial-hypertension-compared-to-idiopathic-pulmonary-arterial-hypertension/. Accessed .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/n-terminal-pro-brain-natriuretic-peptide-is-disproportionately-elevated-in-scleroderma-associated-pulmonary-arterial-hypertension-compared-to-idiopathic-pulmonary-arterial-hypertension/