Pulmonary hypertension in sarcoidosis: A review

Corte, Tamera J.; Wells, Adrian; Nicholson, Andrew G.; Hansell, David M.; Wort, Stephen J.

doi:10.1111/j.1440-1843.2010.01872.x

Cited by 105 publications

(81 citation statements)

References 55 publications

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“…20,21 The mechanisms linking both intermittent nocturnal and exercise oxygen desaturation with survival may be related to the pathogenetic role of pulmonary hypoxic vasoconstriction in the development or worsening of pulmonary arterial hypertension (PH) disproportionate to the severity of the underlying IPF. 22 Several studies have shown that PH and its markers (such as pulmonary vascular resistance) and markers of cardiac stress (such as the levels of brain natriuretic peptide) predict survival in IPF. 23,24 In this study, both oxygen desaturation indices investigated were correlated with the RVSP measured with Doppler echocardiography, thus providing the link between intermittent oxygen desaturation and pulmonary hypertension as well as its associated mortality.…”

Section: Discussionmentioning

confidence: 99%

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Kolilekas

Manali

Vlami

et al. 2013

Journal of Clinical Sleep Medicine

122

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background: Recent studies suggest poor sleep quality in patients with idiopathic pulmonary fi brosis (IPF). However, so far, the impact of IPF-related sleep breathing disorders (SBDs) on survival has not been extensively studied. Methods: In a cohort of 31 (24 males) treatment-naïve, newly diagnosed consecutive IPF patients, we prospectively investigated the relationship of SBD parameters such as apnea-hypopnea index (AHI), maximal difference in oxygen saturation between wakefulness and sleep (maxdiff SpO 2 ), and lowest sleep oxygen saturation (lowest SpO 2 ) with clinical (survival, dyspnea, daytime sleepiness), pulmonary function, submaximal (6-min walk test [6MWT]) and maximal exercise variables (cardiopulmonary exercise test [CPET]), and right ventricular systolic pressure (RVSP). Results: Sleep oxygen desaturation exceeded signifi cantly that of maximal exercise (p < 0.001). Maxdiff SpO 2 was inversely related to survival, DLCO%, and SpO 2 after 6MWT, and directly with dyspnea, AHI, and RVSP. The lowest SpO 2 was directly related to survival and to functional (TLC%, DLCO%) as well as submaximal and maximal exercise variables (6MWT

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Section: Discussionmentioning

confidence: 99%

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Kolilekas

Manali

Vlami

et al. 2013

Journal of Clinical Sleep Medicine

122

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“…However, PH may also occur in milder disease. Since there may be key differences in pathogenesis and clinical implications of PH between the less severe forms of disease and advanced fibrosis, it has been proposed to distinguish between the two ''stages'' of PH in IPF: PH secondary to underlying lung disease; and disproportionate PH [21]. IPF patients should be evaluated for PH when: 1) symptoms are more severe than those expected based on lung function data (dyspnoea and fatigue are symptoms of IPF as well as PH); 2) signs of right heart failure develop; and 3) clinical deterioration is not matched by the decline in pulmonary function.…”

Section: Diagnosis Of Ph In Ipfmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

European Respiratory Review

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Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed “out-of-proportion” PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs

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“…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).…”

mentioning

confidence: 99%

Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease and Pulmonary Fibrosis: Prevalence and Hemodynamic Differences in Lung Transplant Recipients at Transplant Center's Referral Time

Solidoro

Patrucco

Bonato

et al. 2015

Transplantation Proceedings

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Introduction. Single or bilateral lung transplantation is a therapeutic procedure for endstage 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.

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Pulmonary hypertension in sarcoidosis: A review

Cited by 105 publications

References 55 publications

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Sleep Oxygen Desaturation Predicts Survival in Idiopathic Pulmonary Fibrosis

Pulmonary hypertension in chronic interstitial lung diseases

Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease and Pulmonary Fibrosis: Prevalence and Hemodynamic Differences in Lung Transplant Recipients at Transplant Center's Referral Time

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