Pulmonary Hypertension as a Prognostic Indicator at the Initial Evaluation in Idiopathic Pulmonary Fibrosis

Kimura, Motohiro; Taniguchi, Hiroyuki; Kondoh, Yasuhiro; Kimura, Tomoki; Kataoka, Kensuke; Nishiyama, Osamu; Aso, Hirotomo; Sakamoto, Koji; Hasegawa, Yoshinori

doi:10.1159/000345221

Cited by 190 publications

(218 citation statements)

References 37 publications

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“…Our study showed that 35% of IPF patients had high TRGP and demonstrated that TRPG was an independent prognostic factor for disease and patient outcomes. A previous study conducted to support the validity of our results was reported by Kimura et al 18 Under the evaluation of 101 mild IPF patients (mean %FVC 70.2±20.1%) undergoing RHC, they showed that 35% of the patients had a mean pulmonary artery pressure (m-PAP)>20 mmHg and suggested that m-PAP is an independent prognostic factor. PH is considered to be present even in mild stages in IPF patients.…”

Section: Pulmonary Research and Respiratory Medicinesupporting

confidence: 88%

Serial Measurements of Tricuspid Regurgitation Pressure Gradient by Echocardiography Predict Prognosis In Idiopathic Pulmonary Fibrosis

Kitamura

Otsuka²,

Yamada³

et al. 2016

Pulm Res Respir Med Open J

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Background and Objectives: Idiopathic Pulmonary Fibrosis (IPF), especially with emphysema, reportedly involved with Pulmonary Hypertension (PH). However, it is not elucidated whether pulmonary arterial pressure changes serially during the course and influence on prognosis in IPF. We examined whether serial measurements of Tricuspid Regurgitation Pressure Gradient (TRPG) by echocardiography were meaningful predictors of IPF patient survival. Methods: We retrospectively investigated 83 IPF patients. The echocardiographic TRPG cutoff was set at 30 mmHg, and the subjects were divided into two groups: high TRPG and normal TRPG. We also evaluated the relationship between serial TRPG changes during follow-up. Results: A total of 28 patients were included in the high TRPG group. The high TRPG group showed significantly lower %FVC and %DLco, higher A-aDO 2 , shorter 6-minute walk test distance, and more frequent emphysema than the normal TRPG group. The high TRPG group also had poorer survival than the normal TRPG group. A multivariate Cox proportional hazard model demonstrated that TRPG, %FVC, and A-aDO 2 significantly affected patient survival. Thirty-six patients underwent echocardiography twice. At the time of the second echocardiography, 7 patients with normal TRPG at baseline (n=22) increased to a TRPG of more than 30 mmHg. These patients had significantly showed poorer survival. Conclusions: TRPG is an independent prognostic factor in IPF. Emphysema involvement, decreased DLco, and decreased FVC were associated with an increase in TRPG. Serial measurements of TRPG are recommended for the early detection of PH and predict prognosis in IPF patients.

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Section: Pulmonary Research and Respiratory Medicinesupporting

confidence: 88%

Serial Measurements of Tricuspid Regurgitation Pressure Gradient by Echocardiography Predict Prognosis In Idiopathic Pulmonary Fibrosis

Kitamura

Otsuka²,

Yamada³

et al. 2016

Pulm Res Respir Med Open J

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“…In general, the prevalence of PH was similar in studies from Europe [18,25,[40][41][42][43][44][45][47][48][49], North America [5,6,26,28,46,[50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65][66][67], Asia and Africa [37,[68][69][70][71][72][73][74][75], although the majority of evidence was reported from North America (n=23, 53%). Across studies, the prevalence of PH among IPF patients ranged from 3% to 86%; most estimates tended to be between 30% and 50% (figure 3) [25, 43, 44, 47, 50, 52-66, 68, 71-73].…”

Section: Pulmonary Hypertensionmentioning

confidence: 84%

“…A Kaplan-Meier curve revealed significantly worse survival among patients whose mPAP was >20 mmHg than among those whose mPAP was ⩽20 mmHg (log-rank test p=0.001). The median survival estimates were 20.8 and 37.5 months, respectively [74].…”

Section: Pulmonary Hypertensionmentioning

confidence: 98%

“…Data from 210 IPF patients identified from a hospital database in China found that the mean survival time in patients with and without sPAP ⩾37 mmHg was 24 versus 57 months, respectively ( p=0.008); in the multivariable analysis, PH was associated with a significantly increased mortality risk (risk ratio 3.393, 95% CI 1.444-7.973; p=0.005) [37]. mPAP was also independently, significantly, associated with 5-year mortality (risk ratio 1.064, 95% CI 1.015-1.116; p=0.010) in an analysis of 101 IPF patients from a hospital in Japan [74]. A receiver operating curve analysis found that a value of 20 mmHg was the optimal (area under the curve 0.679, sensitivity 55.0%, specificity 75.4%) cut-off.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

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Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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Idiopathic pulmonary fibrosis (IPF) is associated with a fatal prognosis and manifests in patients over 60 years old who may have comorbidities. The prevalence and impact of comorbidities on the clinical course of IPF is unclear.This systematic literature review examined the prevalence of comorbidities and mortality associated with comorbidities in IPF patients. Relevant observational studies published in English from January 1990 to January 2015 identified via MEDLINE and EMBASE were included; bibliographies of articles were also searched.Among the 126 studies included, prevalence of pulmonary hypertension (PH) was 3-86%, 6-91% for obstructive sleep apnoea, 3-48% for lung cancer and 6-67% for chronic obstructive pulmonary disease (COPD). Nonrespiratory comorbidities included ischaemic heart disease (IHD) (3-68%) and gastrooesophageal reflux (GER) (0-94%). Mortality was highest among patients with IPF and lung cancer. Most studies assessed relatively small samples of patients with IPF.PH, COPD, lung cancer, GER and IHD are significant comorbidities; differences in IPF severity, case definitions and patient characteristics limited the comparability of findings. The identification and prompt treatment of comorbidities may have a clinically significant impact on overall outcome that is meaningful for patients with IPF. @ERSpublications Identification and prompt treatment of comorbidities has a clinically significant impact on outcome for IPF patients

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“…PATEL et al [16] observed that PH (mean PAP .25 mmHg) was an independent predictor of mortality in 376 patients with IPF referred for lung transplantation. In a recent study, KIMURA et al [32] confirmed by multivariate analysis that a high mean PAP value measured at the initial evaluation of IPF patients undergoing RHC is an independent predictor of survival. Unlike many other studies [1,33,34], this retrospective analysis focused on IPF patients affected by milder disease (mean FVC 70.2%, and mean DLCO 47.9%) [32].…”

Section: Prognostic Significance Of Ph In Ipfmentioning

confidence: 89%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

Eur Respir Rev

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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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Pulmonary Hypertension as a Prognostic Indicator at the Initial Evaluation in Idiopathic Pulmonary Fibrosis

Cited by 190 publications

References 37 publications

Serial Measurements of Tricuspid Regurgitation Pressure Gradient by Echocardiography Predict Prognosis In Idiopathic Pulmonary Fibrosis

Serial Measurements of Tricuspid Regurgitation Pressure Gradient by Echocardiography Predict Prognosis In Idiopathic Pulmonary Fibrosis

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

Pulmonary hypertension in chronic interstitial lung diseases

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