Systemic sclerosis
Natriuretic Peptides in Systemic Sclerosis-related Pulmonary Arterial Hypertension

https://doi.org/10.1016/j.semarthrit.2009.03.005Get rights and content

Objectives

Systemic sclerosis-related pulmonary arterial hypertension (SSc PAH) is a major complication of both limited and diffuse systemic sclerosis and leads to substantial morbidity and mortality. Natriuretic peptides (NP) are clinically useful markers of right ventricular dysfunction and pulmonary hypertension. The aim of our review was to examine the evidence for the physiologic, diagnostic, and prognostic role of NP in the context of SSc PAH.

Methods

A Medline search for articles published between January 1999 and December 2008 was conducted using the following keywords: natriuretic peptides, systemic sclerosis or scleroderma, and pulmonary arterial hypertension.

Results

In patients with SSc PAH, NP levels increase in proportion to the extent of right ventricular dysfunction and correlate significantly with functional capacity and echocardiographic and hemodynamic parameters. NP may also provide prognostic information beyond conventional risk markers but their use has to be considered against the background of the parameters that may influence their concentration.

Conclusion

There is growing evidence that NP, along with the traditional assessment modalities such as echocardiography and the 6-minute walking test, may be a suitable marker for SSc PAH in terms of screening, diagnostic evaluation, risk stratification, and response to therapy; this merits prospective evaluation.

Section snippets

Methods

To outline the role of NP in patients with SSc PAH, we performed an extensive internet search (PubMed) using the following keywords: natriuretic peptides, systemic sclerosis or scleroderma, and pulmonary arterial hypertension. The present study searched Medline for articles published between January 1998 and December 2008 related to this topic. Articles were selected if they had been published as full journal articles in English (abstracts, poster presentations, conference proceedings, or

Results

The literature search as described in Methods highlighted a number of published studies. These are presented later in this review, focusing on the value of the NP in the detection and risk stratification of SSc PAH. First, biology and assays of NP are briefly described.

Pathogenetic Mechanisms and NP Release in SSc PAH

Activation of neurohormonal systems with vasoconstrictor, antidiuretic, proinflammatory, hypertrophic, and cytoproliferative effects have been detected in SSc patients (40). While the activation of vasoconstrictor hormones (plasma catecholamine, renin-angiotensin-aldosterone system) represents a compensatory mechanism at least in the early phase of the disease, RV involvement determined by various degrees of pulmonary hypertension is a powerful stimulus for eliciting secretion of cardiac NP.

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    The first 2 authors contributed equally to this work.

    The authors have no conflicts of interest to disclose.

    Dr Giannakoulas has received training and research grants from the Hellenic Cardiological Society, the Hellenic Heart Foundation, and the DG Education & Culture–LLP Programme–Leonardo Da Vinci Mobility. Professor Gatzoulis and the Royal Brompton Adult Congenital Heart Centre and Centre for Pulmonary Hypertension have received support from the British Heart Foundation.

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