Pulmonary Hypertension in Patients With Interstitial Lung Diseases

Ryu, Jay H.; Krowka, Michael J.; Pellikka, Patricia A.; Swanson, Karen L.; McGoon, Michael D.
March 2007
Mayo Clinic Proceedings;Mar2007, Vol. 82 Issue 3, p342
Academic Journal
Pulmonary hypertension (PH) In patients with interstitial lung diseases (ILDs) is not well recognized and can occur in the absence of advanced pulmonary dysfunction or hypoxemia. To address this topic, we identified relevant studies in the English language by searching the MEDLINE database (1966 to November 2006) and by individually reviewing the references of identified articles. Connective tissue disease-related ILD, sarcoidosis, idiopathic pulmonary fibrosis, and pulmonary Langerhans cell histiocytosis are the ILDs most commonly associated with PH. Pulmonary hypertension Is an underrecognized complication in patients with ILDs and can adversely affect symptoms, functional capacity, and survival. Pulmonary hypertension can arise In patients with ILDs through various mechanisms, including pulmonary vasoconstriction and vascular remodeling, vascular destruction associated with progressive parenchymal fibrosis, vascular inflammation, perivascular fibrosis, and thrombotic angiopathy. Diagnosis of PH in these patients requires a high Index of suspicion because the clinical presentation tends to be nonspecific, particularly in the presence of an underlying parenchymal lung disease. Doppler echocardiography is an essential tool in the evaluation of suspected PH and allows ready recognition of cardiac causes. Right heart catheterization is needed to confirm the presence of PH, assess its severity, and guide therapy. Management of PH In patients with ILDs is guided by identification of the underlying mechanism and the clinical context. An increasing number of available pharmacologic agents in the treatment of PH allow possible treatment of PH in some patients with ILDs. Whether specific treatment of PH in these patients favorably alters functional capacity or outcome needs to be determined.


Related Articles

  • Pulmonary survival study in 91 patients with systemic sclerosis. Poormoghim, Hadi; Lakeh, Maziar; Mohammadipour, Mastoureh; Talehy-Moineddin, Shima; Sodagari, Faezeh // Rheumatology International;Dec2011, Vol. 31 Issue 12, p1577 

    In systemic sclerosis (SSc), major determinant of morbidity and mortality is pulmonary complication including pulmonary interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). In this study, the natural course of pulmonary involvement in SSc patients was investigated. This was...

  • Lung volumes in interstitial lung disease in smokers. Macnair, Archie // Thorax;Jan2012, Vol. 67 Issue 1, p48 

    The article discusses the results of a study which found that patients with defined chronic obstructive pulmonary disease (COPD) and interstitial lung changes are more likely to have decreased emphysematous changes and total lung capacity compared to those with only interstitial lung disease.

  • Statin Use Is Associated with Reduced Mortality in Patients with Interstitial Lung Disease. Vedel-Krogh, Signe; Nielsen, Sune F.; Nordestgaard, Børge G. // PLoS ONE;10/16/2015, Vol. 10 Issue 10, p1 

    Introduction: We hypothesized that statin use begun before the diagnosis of interstitial lung disease is associated with reduced mortality. Methods: We studied all patients diagnosed with interstitial lung disease in the entire Danish population from 1995 through 2009, comparing statin use...

  • Novel equation effective at predicting survival in pulmonary arterial hypertension.  // Cardiology Today;Feb2010, Vol. 13 Issue 2, p16 

    The article discusses research on the efficacy of a new equation in predicting survival in patients with pulmonary arterial hypertension compared with the equation traditionally used by the National Institutes of Health, with a reference to a study by T. Thenappan that was published in an issue...

  • RECENT TRENDS IN INTERSTITIAL LUNG DISEASE: STUDY OF 25 CASES. Palat, Paltial; Parmar, Hiren D. // International Journal of Medical Science & Public Health;2013, Vol. 2 Issue 1, p52 

    Background: Interstitial lung disease accounts for more than 200 etiology. There has been worldwide increase in diagnosis of interstitial lung disease because of the help of recent advance diagnostic tools. Aims & Objective: to study age & sex distribution, to correlate symptoms & pulmonary...

  • Validity of the Saint George's Respiratory Questionnaire in the evaluation of the health-related quality of life in patients with interstitial lung disease secondary to systemic sclerosis. A. Lemos // Rheumatology;Feb2007, Vol. 46 Issue 2, p296 

    Objectives. Interstitial lung disease (ILD) profoundly affects the health-related quality of life (HRQoL) in patients with systemic sclerosis (SSc). We tested the validity of the Saint George's Respiratory Questionnaire (SGRQ), a lung-specific HRQoL-evaluation tool, in a population of SSc...

  • Erasmus Syndrome in a 42-Year-Old Male: A Rare Case Report. CHAKRABARTI, SUBRATA; PAN, KOUSHIK // Journal of Clinical & Diagnostic Research;May2015, Vol. 9 Issue 5, p1 

    Erasmus syndrome is a rare entity in which systemic sclerosis develops following exposure to silica with or without silicosis. Few case reports are available in literature. We report here a case of Erasmus syndrome in a 42-year-old manual labourer. The patient presented with arthralgia,...

  • Human Herpesvirus 8 and Pulmonary Hypertension. Nicastri, Emanuele; Vizza, Carmine Dario; Carletti, Fabrizio; Cicalini, Stefania; Badagliacca, Roberto; Poscia, Roberto; Ippolito, Giuseppe; Fedele, Francesco; Petrosillo, Nicola // Emerging Infectious Diseases;Sep2005, Vol. 11 Issue 9, p1480 

    Human herpesvirus 8 (HHV-8) antibodies were detected in 1 of 33 patients with pulmonary hypertension (including in 1 of 16 with idiopathic pulmonary arterial hypertension), 5 of 29 with cystic fibrosis. and 3 of 13 with interstitial lung disease. No relationship between HHV-8 infection and...

  • Lung scintigraphy with nonspecific human immunoglobulin G (99mTc-HIG) in the evaluation of pulmonary involvement in connective tissue diseases: correlation with pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT). Kostopoulos, Ch.; Koutsikos, J.; Toubanakis, C.; Moulopoulos, L. A.; Mamoulakis, Ch.; Gialafos, E.; Sfikakis, P. P.; Zerva, Ch.; Mavrikakis, M.; Leondi, A. // European Journal of Nuclear Medicine & Molecular Imaging;Feb2008, Vol. 35 Issue 2, p343 

    In patients with connective tissue diseases (CTD), the early detection and evaluation of the severity of the pulmonary involvement is mandatory. High-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) are considered to be valuable noninvasive diagnostic modalities....


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics