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: 1.17). 07/2009; 26(1):7-19.
Source: PubMed


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.

Download full-text


Available from: Tamera J Corte, Oct 07, 2015
1 Follower
46 Reads
  • Source
    • "Rapid progression of PH was reported in latestage diffuse parenchymal lung disease (DPLD)/IPF patients [12]. In some studies, the prognosis of PH in lung fibrosis is not linked to the mPAP values but to pulmonary vascular resistance [13] or cardiac index (CI), with CI values < 2.4 L/min/m 2 correlated to survival of only a few months [14]. Assessment and definition of PH due to chronic lung diseases is a fundamental step in end-stage lung diseases (Group 3). "
    [Show abstract] [Hide abstract]
    ABSTRACT: Introduction: Single or bilateral lung transplantation is a therapeutic procedure for end-stage lung diseases. In particular, in cases of chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis, patients can be referred to the transplant center late and with important comorbilities. Pulmonary hypertension (PH) associated with lung diseases not only is an index of poor outcome but also is an indication for bilateral procedure. Methods: We conducted a retrospective observational study. We analyzed right heart catheterization in a consecutive series of patients who underwent lung transplantation from 2006 to 2014 for end-stage COPD and pulmonary fibrosis. Results: We included in the study 73 patients (35 with fibrosis and 38 with COPD); prevalence of PH was higher in the COPD group (84.3% vs 31.4%), and with worse hemodynamic parameters (mean pulmonary artery pressure [30.3 mm Hg vs 24.1 mm Hg]). The majority of COPD patients presented mild or moderate PH, and fibrosis patients showed normal pulmonary arterial pressures. Conclusions: COPD patients are referred to the Transplant Center with a higher prevalence of PH because of an echocardiographic screening or a late referral, but many patients survive on the waiting list and undergo the procedure. On the other hand, patients transplanted with interstitial diseases have a lower prevalence of PH; this can be explained by an earlier referral or a higher mortality on the waiting list and a more aggressive and rapidly progressing disease.
    Transplantation Proceedings 09/2015; 47(7):2161-5. DOI:10.1016/j.transproceed.2015.01.031 · 0.98 Impact Factor
  • Source
    • "Genetic associations with the disease include pulmonary surfactant-associated proteins (SFTPA-1 and SFTPA-2), telomerase reverse transcriptase (TERT), and telomerase RNA component (TERC) [119, 120]. It is of interest that statistically significant association in survival has been reported between IPF patients with and without PH at the time of initial IPF diagnosis [121]; PH in IPF can develop either as consequence of the fibrotic process or disproportionate to the degree of fibrotic lung damage [122]. Although chronic hypoxia and its subsequent pulmonary arterial vasoconstriction are thought to have a major role in secondary IPF-PH, studies that showed the existence of PH in such patients even with arterial pO2 levels within normal range (normoxic) led the investigators to partly relinquish this concept and redirect to other possible underlying mechanisms [123–125]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Idiopathic pulmonary arterial hypertension (IPAH) has been extensively investigated, although it represents a less common form of the pulmonary hypertension (PH) family, as shown by international registries. Interestingly, in types of PH that are encountered in parenchymal lung diseases such as interstitial lung diseases (ILDs), chronic obstructive pulmonary disease (COPD), and many other diffuse parenchymal lung diseases, some of which are very common, the available data is limited. In this paper, we try to browse in the latest available data regarding the occurrence, pathogenesis, and treatment of PH in chronic parenchymal lung diseases.
    Pulmonary Medicine 10/2012; 2012(20):684781. DOI:10.1155/2012/684781
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: ADVERTIMENT. La consulta d'aquesta tesi queda condicionada a l'acceptació de les següents condicions d'ús: La difusió d'aquesta tesi per mitjà del servei TDX ( ha estat autoritzada pels titulars dels drets de propietat intel·lectual únicament per a usos privats emmarcats en activitats d'investigació i docència. No s'autoritza la seva reproducció amb finalitats de lucre ni la seva difusió i posada a disposició des d'un lloc aliè al servei TDX. No s'autoritza la presentació del seu contingut en una finestra o marc aliè a TDX (framing). Aquesta reserva de drets afecta tant al resum de presentació de la tesi com als seus continguts. En la utilització o cita de parts de la tesi és obligat indicar el nom de la persona autora. ADVERTENCIA. La consulta de esta tesis queda condicionada a la aceptación de las siguientes condiciones de uso: La difusión de esta tesis por medio del servicio TDR ( ha sido autorizada por los titulares de los derechos de propiedad intelectual únicamente para usos privados enmarcados en actividades de investigación y docencia. No se autoriza su reproducción con finalidades de lucro ni su difusión y puesta a disposición desde un sitio ajeno al servicio TDR. No se autoriza la presentación de su contenido en una ventana o marco ajeno a TDR (framing). Esta reserva de derechos afecta tanto al resumen de presentación de la tesis como a sus contenidos. En la utilización o cita de partes de la tesis es obligado indicar el nombre de la persona autora. WARNING. On having consulted this thesis you're accepting the following use conditions: Spreading this thesis by the TDX ( service has been authorized by the titular of the intellectual property rights only for private uses placed in investigation and teaching activities. Reproduction with lucrative aims is not authorized neither its spreading and availability from a site foreign to the TDX service. Introducing its content in a window or frame foreign to the TDX service is not authorized (framing). This rights affect to the presentation summary of the thesis as well as to its contents. In the using or citation of parts of the thesis it's obliged to indicate the name of the author.
Show more