Article

Pulmonary hypertension in idiopathic pulmonary fibrosis: a review.

Department of Thoracic Medicine Royal Brompton Hospital, London, UK.
Sarcoidosis, vasculitis, and diffuse lung diseases: official journal of WASOG / World Association of Sarcoidosis and Other Granulomatous Disorders (Impact Factor: 1.74). 07/2009; 26(1):7-19.
Source: PubMed

ABSTRACT Pulmonary hypertension (PH) is a common in patients with idiopathic pulmonary fibrosis (IPF) referred for transplantation. When present, PH is associated with increased mortality, and may explain the deterioration of some patients with preserved pulmonary function. PH in IPF may develop as a consequence of, or disproportionate to the underlying fibrotic lung disease. The distinction between these two 'stages' of PH is essential as there are key differences in their pathophysiology, identification, and potential treatment options. Treatment advances in idiopathic pulmonary artery hypertension have focused attention on PH associated with underlying lung disease. We focus on pathogenetic mechanisms, identification of PH, and the potential for therapeutic intervention for PH in IPF. Although vascular ablation, and chronic hypoxia are both important in the aetiology of secondary PH, these mechanisms do not explain the development of disproportionate PH. In these patients, the early development of PH may be associated with increased fibrotic cell mediators, abnormal vasculature or response to hypoxia, seen in IPF. Nocturnal and exercise desaturation are common in IPF, and may precede and contribute to the development PH. Therapeutic options for PH in IPF are limited, and there have been no controlled trials. Successful therapeutic intervention in pulmonary arterial hypertension, has led to suggestions that therapeutic intervention with PH specific therapy may be useful. However, controlled trials are warranted before therapy can be recommended. In the design of such trials, the distinction between secondary and disproportionate PH is essential.

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    ABSTRACT: Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation. The task force encompassing the authors of this article provided criteria for this discrimination and suggested using the following definitions for group 3 patients, as exemplified for COPD, IPF, and CPFE: COPD/IPF/CPFE without PH (mean pulmonary artery pressure [mPAP] <25 mm Hg); COPD/IPF/CPFE with PH (mPAP ≥25 mm Hg); PH-COPD, PH-IPF, and PH-CPFE); COPD/IPF/CPFE with severe PH (mPAP ≥35 mm Hg or mPAP ≥25 mm Hg with low cardiac index [CI <2.0 l/min/m(2)]; severe PH-COPD, severe PH-IPF, and severe PH-CPFE). The "severe PH group" includes only a minority of chronic lung disease patients who are suspected of having strong general vascular abnormalities (remodeling) accompanying the parenchymal disease and with evidence of an exhausted circulatory reserve rather than an exhausted ventilatory reserve underlying the limitation of exercise capacity. Exertional dyspnea disproportionate to pulmonary function tests, low carbon monoxide diffusion capacity, and rapid decline of arterial oxygenation upon exercise are typical clinical features of this subgroup with poor prognosis. Studies evaluating the effect of pulmonary arterial hypertension drugs currently not approved for group 3 PH patients should focus on this severe PH group, and for the time being, these patients should be transferred to expert centers for individualized patient care.
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