Pulmonary hypertension in interstitial lung disease

Abstract

In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs.

Analysis of V/Q-matching—a safety “biomarker” in pulmonary drug development?

“…NPAHPH forms with underlying lung disease, e.g. COPD, ILD; Ryu et al, 2007;Behr and Ryu, 2008;Barberá and Blanco, 2009), a similar reasoning applies. Table 1.…”

Interstitial lung disease: new challenges and evolving phenotypes

“…Two main challenges in IPF are related to situations that significantly contribute to the mortality of this devastating disease, namely acute exacerbations of disease [12] and severe pulmonary hypertension [13]. The former is defined by the rapid deterioration of IPF during the course of disease that cannot be attributed to an identified cause.…”

A message on the lips

“…Pulmonologists will encounter three main manifestations of HHT: pulmonary arterial hypertension; high cardiac output failure in the setting of large AVMs in the liver; and pulmonary AVMs. A forthcoming article by FAUGHNAN et al[2], as part of the European Respiratory Journal's (ERJ) current series on pulmonary hypertension [2][3][4][5][6][7], will review the cardiopulmonary manifestations of HHT in detail.Pulmonary arterial hypertension and liver AVMs are predominantly seen in patients with Alk-1 mutations (also called HHT type 2) [8]. Isolated pulmonary arterial hypertension in patients with HHT is clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension, a disease that has been linked to mutations in another TGF-b superfamily receptor, termed bone morphogenetic protein receptor-II [9].…”

“…[2], as part of the European Respiratory Journal's (ERJ) current series on pulmonary hypertension [2][3][4][5][6][7], will review the cardiopulmonary manifestations of HHT in detail.…”

“…Pulmonologists will encounter three main manifestations of HHT: pulmonary arterial hypertension; high cardiac output failure in the setting of large AVMs in the liver; and pulmonary AVMs. A forthcoming article by FAUGHNAN et al [2], as part of the European Respiratory Journal's (ERJ) current series on pulmonary hypertension [2][3][4][5][6][7], will review the cardiopulmonary manifestations of HHT in detail.…”