How does comorbidity influence survival in idiopathic pulmonary fibrosis?

Hyldgaard, Charlotte; Hilberg, Ole; Bendstrup, Elisabeth

doi:10.1016/j.rmed.2014.01.008

Cited by 135 publications

(150 citation statements)

References 30 publications

(32 reference statements)

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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: 85%

“…Presence of PH was found to be associated with an increased risk for death (HR for death, PH versus no PH 4.03, 95% CI 1.17-27.9; p=0.03 (study population was CPFE patients) [76]; HR 3.6, 95% CI not reported; p=0.0001 [44]). However, an analysis of data from 121 IPF patients from a hospital in Denmark did not find a significant association between PH and mortality, although a numerical trend remained after adjustment for age, gender and forced vital capacity (FVC) (HR 2.2, 95% CI 0.94-5.2; p=0.068) [48]; similarly, a review of IPF patients evaluated for lung transplantation at a US university hospital did not find a significant association between mean pulmonary artery pressure (mPAP) and mortality [67]. Among a review based on existing data of 25 IPF patients from the USA with PH, 1-year mortality was 29% [57].…”

Section: Pulmonary Hypertensionmentioning

confidence: 97%

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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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Section: Pulmonary Hypertensionmentioning

confidence: 85%

Section: Pulmonary Hypertensionmentioning

confidence: 97%

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

et al. 2015

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“…In the literature, the prevalence of PH in patients affected by IPF ranges from 8% to 21%, but higher percentages (30% to 50%) are found in advanced and endstage cases [19,26]. Among those patients, a few may present with mPAP values >40 mm Hg [7].…”

Section: Discussionmentioning

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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“…Le comorbidità più frequenti ed importanti associate ad IPF sono: il reflusso gastro-esofageo (RGE), le malattie cardiovascolari, il cancro del polmone, la depressione, i disturbi respiratori del sonno, il sottopeso, il diabete, l'ipertensione polmonare e l'enfisema [24,25]. Le conseguenze di ognuna di queste comorbidità sulla storia e la prognosi dell'IPF non sono ancora del tutto chiarite, ma l'ipertensione polmonare, il cancro del polmone, oltre all'enfisema combinato alla IPF (Sindrome CPFE) hanno un documentato impatto negativo sulla sopravvivenza [26,27].…”

Section: Comorbiditàunclassified

“…La prevalenza di enfisema alla diagnosi oscilla tra il 30% e il 47% e questi pazienti sembrano avere una prognosi peggiore rispetto a quelli che hanno solo IPF. La prognosi è certamente peggiore se si associa anche ipertensione polmonare [24,[47][48][49][50][51]. Dati disponibili di prevalenza di Ipertensione Polmonare Precapillare (PH) in corso di IPF variano dal 31% all'85% [26,[52][53][54][55][56].…”

Section: Comorbiditàunclassified

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

Belisari¹,

Bettoni

Cortesi

et al. 2017

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To date, there are few therapeutic answers for Idiopathic pulmonary fibrosis (IPF) and only two pharmacological treatments have a marketing authorization for this disease. Recently nintedanib (Ofev®) has been authorized as a new therapeutic option and its economic profile has been evaluated by international Health Technology Assessment (HTA) bodies. IPF has important implications for everyday life of patients and their carers, negatively influencing their quality of life and bringing heavy economic burden to the NHS and to the entire society. It is, therefore important to consider these aspects for the Italian environment and to perform a pharmacoeconomic evaluation to define the efficiency of nintedanib in IPF by means of a Cost-Utility Analysis (CUA). As IPF is a chronic and progressive disease, a lifetime Markov model has been therefore developed with health states describing the patient’s condition as a combination of lung function and exacerbation history. The cohort entered in the model at different Forced Vital Capacity (FVC%) predicted health states, without exacerbation. The Clinical data used to perform this CUA were derived from clinical trials and the relative efficacy of nintedanib versus the comparator (pirfenidone) was then obtained from a Network Meta-Analysis (NMA) combining data reported in each primary study (INPULSIS 1-2 and TOMORROW trials for nintedanib, and CAPACITY and ASCEND trials for pirfenidone, respectively). At base-case, treatment with nintedanib resulted in a slightly lower estimated total cost vs pirfenidone, better safety profile and lower risk of acute exacerbations with an advantage in Quality Adjusted Life years (QALYs) gained. These results were confirmed by the sensitivity analysis. Although nintedanib appears to be a valuable option for the NHS to treat IPF patients, future data evidence, as long-term or real-life data, will help to confirm these results.[In Italian]

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How does comorbidity influence survival in idiopathic pulmonary fibrosis?

Cited by 135 publications

References 30 publications

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review

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

[Nintedanib for the treatment of idiopathic pulmonary fibrosis in Italy]

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