The inclusion of N-terminal pro-brain natriuretic peptide in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension: a cohort study

Abstract

IntroductionPulmonary arterial hypertension (PAH) is a major cause of mortality in systemic sclerosis (SSc). Screening guidelines for PAH recommend multiple investigations, including annual echocardiography, which together have low specificity and may not be cost-effective. We sought to evaluate the predictive accuracy of serum N-terminal pro-brain natriuretic peptide (NT-proBNP) in combination with pulmonary function tests (PFT) (‘proposed’ algorithm) in a screening algorithm for SSc-PAH.MethodsWe evaluated our proposed algorithm (PFT with NT-proBNP) on 49 consecutive SSc patients with suspected pulmonary hypertension undergoing right heart catherisation (RHC). The predictive accuracy of the proposed algorithm was compared with existing screening recommendations, and is presented as sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV).ResultsOverall, 27 patients were found to have pulmonary hypertension (PH) at RHC, while 22 had no PH. The sensitivity, specificity, PPV and NPV of the proposed algorithm for PAH was 94.1%, 54.5%, 61.5% and 92.3%, respectively; current European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines achieved a sensitivity, specificity, PPV and NPV of 94.1%, 31.8%, 51.6% and 87.5%, respectively. In an alternate case scenario analysis, estimating a PAH prevalence of 10%, the proposed algorithm achieved a sensitivity, specificity, PPV and NPV for PAH of 94.1%, 54.5%, 18.7% and 98.8%, respectively.ConclusionsThe combination of NT-proBNP with PFT is a sensitive, yet simple and non-invasive, screening strategy for SSc-PAH. Patients with a positive screening result can be referred for echocardiography, and further confirmatory testing for PAH. In this way, it may be possible to shift the burden of routine screening away from echocardiography. The findings of this study should be confirmed in larger studies.

Contribution of Speckle Tracking to Estimation of Pulmonary Hypertension by Standard Doppler Echocardiography in Patients with Systemic Sclerosis and MCTD

“…Thus, NT-proBNP and DLCO now belong to the most useful predictors of pulmonary hypertension in SSc patients [20,21]. Regarding differences between limited or diffuse SSc and MCTD in the present study, patients with MCTD were significant younger than SSc patients and patients with diffuse SSc had significant lower total lung capacity and DLCO than those with limited SSc or MCTD.…”

Recent Advances in Systemic Scleroderma in Childhood

“…All these markers signify risks for certain organ involvement but do not fluctuate according to the level of the activity of the disease. The above mentioned pro-BNP is an accepted marker of myocardial stress in PAH monitoring [86], which is supported by pediatric studies [87][88][89]. Several novel biomarkers of the disease have recently been reported, with most of them relating to fibrosis pathogenesis.…”

Use of ECG and Other Simple Non-Invasive Tools to Assess Pulmonary Hypertension

“…In different clinical settings, various NT-proBNP thresholds may be clinically relevant. In the DETECT study [6], a continuous scale, but no single threshold for NT-proBNP was used, while another recent -PAH screening algorithm in systemic sclerosis recommended a threshold of 210 pg/ml [24]. In the study of Bonderman et al, a low NT-proBNP threshold (80pg/ml) distinguished pre- and post-capillary PH [15].…”