In-Hospital Mortality in Patients with Idiopathic Pulmonary Fibrosis: A US Cohort Study.
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ABSTRACT: Purpose
In patients with idiopathic pulmonary fibrosis (IPF), hospitalizations are associated with high mortality. We sought to determine in-hospital mortality rates and factors associated with in-hospital mortality in patients with IPF.Methods
Patients with IPF were identified from the Premier Healthcare Database, a representative administrative dataset that includes?>?20% of hospital discharges in the US, using an algorithm based on diagnostic codes and billing data. We used logistic regression to analyze associations between patient-, hospital-, and treatment-related characteristics and a composite primary outcome of death during the index visit, lung transplant during the index visit and?>?1 day after admission, or death during a readmission within 90 days.Results
The cohort comprised 6665 patients with IPF hospitalized between October 2011 and October 2014. A total of 963 (14.4%) met the primary outcome. Factors significantly associated with a higher risk of the primary outcome included mechanical ventilation [odds ratio 4.65 (95% CI 3.73, 5.80)], admission to the intensive care unit [1.83 (1.52, 2.21)], treatment with opioids (3.06 [2.57, 3.65]), and a diagnosis of pneumonia [1.44 (1.21, 1.71)]. Factors significantly associated with a lower risk included concurrent chronic obstructive pulmonary disease [0.65 (0.55, 0.77)] and female sex [0.67 (0.57, 0.79)].Conclusions
Patients with IPF, particularly those receiving mechanical ventilation or intensive care, are at substantial risk of death or lung transplant during hospitalization or death during a readmission within 90 days.
SUBMITTER: Durheim MT
PROVIDER: S-EPMC6861436 | biostudies-literature | 2019 Dec
REPOSITORIES: biostudies-literature
Publications
In-Hospital Mortality in Patients with Idiopathic Pulmonary Fibrosis: A US Cohort Study.
Durheim Michael T MT Judy Jennifer J Bender Shaun S Baumer Dorothy D Lucas Joseph J Robinson Scott B SB Mohamedaly Omar O Shah Bimal R BR Leonard Thomas T Conoscenti Craig S CS Palmer Scott M SM
Lung 20190920 6
<h4>Purpose</h4>In patients with idiopathic pulmonary fibrosis (IPF), hospitalizations are associated with high mortality. We sought to determine in-hospital mortality rates and factors associated with in-hospital mortality in patients with IPF.<h4>Methods</h4>Patients with IPF were identified from the Premier Healthcare Database, a representative administrative dataset that includes > 20% of hospital discharges in the US, using an algorithm based on diagnostic codes and billing data. We used lo ...[more]
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Project description:Hospitalizations are common in patients with idiopathic pulmonary fibrosis (IPF) and are associated with high mortality. We used data from the Premier Healthcare Database to determine in-hospital mortality rates and the factors associated with in-hospital mortality in patients with IPF in the era of approved antifibrotic drugs.The Premier Healthcare Database is a detailed and broadly representative database of hospital admissions and discharges in the US. Patients with IPF who were hospitalized between 1 January 2015 and 28 February 2018 were identified using a diagnostic algorithm comprising International Classification of Diseases -9 and International Classification of Diseases -10 diagnostic codes and billing data. Associations between patient-, hospital- and treatment-related factors and a composite outcome of death during the index visit, lung transplant during the index visit but >1 day after admission, or death during a readmission within 90 days of the index visit were analyzed using logistic regression.The cohort comprised 9667 hospitalized patients with IPF. In total, 1414 patients (14.6%) met the composite outcome: 1036 (10.7%) died during the index visit, 371 (3.8%) died during a readmission within 90 days; 7 (0.1%) underwent lung transplant >1 day after admission. Factors significantly associated with a higher risk of the composite outcome included mechanical ventilation (odds ratio 6.41 [95% CI: 5.24, 7.84]), admission to the intensive care unit (1.73 [1.49, 2.00]), attendance by a critical care physician (2.12 [1.33, 3.38]), older age (1.20 [1.12, 1.28] per 10-year increase), and use of intravenous steroids (1.16 [1.00, 1.34]), intravenous antibiotics (1.49 [1.22, 1.83]) and opioids (3.41 [2.95, 3.93]). Factors significantly associated with a lower risk of the composite outcome included female sex (0.70 [0.61, 0.80]), comorbid chronic obstructive pulmonary disease (0.69 [0.60, 0.78]), attendance by a family medicine physician (0.67 [0.48, 0.94]) or internal medicine physician (0.59 [0.46, 0.75]), and use of oral steroids (0.62 [0.51, 0.77]), statins (0.76 [0.67, 0.87]) and proton pump inhibitors (0.80 [0.70, 0.92]).In conclusion, patients with IPF are at risk of mortality during a hospital stay or readmission within 90 days, particularly those who receive mechanical ventilation.
| S-EPMC7676591 | biostudies-literature
Project description:Patients with idiopathic pulmonary fibrosis (IPF) often experience weight loss during the follow-up period. However, the prevalence and clinical impact of weight loss in these patients still need to be elucidated. This retrospective single-center study reviewed 134 consecutive patients diagnosed with IPF. Weight loss of 5% or more over 1 year was defined as significant weight loss. Clinical data of patients were compared according to the significant weight loss. We analyzed whether the clinical impact of significant weight loss differed regarding the pirfenidone dose. The median follow-up period was 22.1 months. The mean age of patients was 67.3 years, and 92.5% were men. Of the 134 patients, 42 (31.3%) showed significant weight loss. Multivariate cox regression analysis revealed that significant weight loss was independently associated with mortality (hazard ratio [HR]; 2.670; 95% confidence interval [CI] 1.099-6.484; p = 0.030) after adjusting for lung function and other significant risk factors (6-min walk test distance: HR, 0.993; 95% CI 0.987-0.998; p = 0.005). The median survival of patients with significant weight loss (n = 22) was relevantly shorter than that of those without significant weight loss (n = 43) in the reduced dose pirfenidone group (28.2 ± 3.3 vs. 43.3 ± 3.2 months, p = 0.013). Compared with patients without significant weight loss (n = 38), patients with significant weight loss (n = 15) also showed a marginally-significant shorter survival in the full-dose pirfenidone group (28.9 ± 3.1 vs. 39.8 ± 2.6 months, p = 0.085). Significant weight loss is a prognostic factor in patients with IPF regardless of pirfenidone dose. Vigilant monitoring might be necessary to detect weight loss during the clinical course in these patients.
| S-EPMC10082839 | biostudies-literature
Project description:BackgroundTwo antifibrotic drugs, pirfenidone and nintedanib, are licensed for the treatment of patients with idiopathic pulmonary fibrosis (IPF). However, there is neither evidence from prospective data nor a guideline recommendation, which drug should be preferred over the other. This study aimed to compare pirfenidone and nintedanib-treated patients regarding all-cause mortality, all-cause and respiratory-related hospitalizations, and overall as well as respiratory-related health care costs borne by the Statutory Health Insurance (SHI).MethodsA retrospective cohort study with SHI data was performed, including IPF patients treated either with pirfenidone or nintedanib. Stabilized inverse probability of treatment weighting (IPTW) based on propensity scores was applied to adjust for observed covariates. Weighted Cox models were estimated to analyze mortality and hospitalization. Weighted cost differences with bootstrapped 95% confidence intervals (CI) were applied for cost analysis.ResultsWe compared 840 patients treated with pirfenidone and 713 patients treated with nintedanib. Both groups were similar regarding two-year all-cause mortality (HR: 0.90 95% CI: 0.76; 1.07), one-year all cause (HR: 1.09, 95% CI: 0.95; 1.25) and respiratory-related hospitalization (HR: 0.89, 95% CI: 0.72; 1.08). No significant differences were observed regarding total (€- 807, 95% CI: €- 2977; €1220) and respiratory-related (€- 1282, 95% CI: €- 3423; €534) costs.ConclusionOur analyses suggest that the patient-related outcomes mortality, hospitalization, and costs do not differ between the two currently available antifibrotic drugs pirfenidone and nintedanib. Hence, the decision on treatment with pirfenidone versus treatment with nintedanib ought to be made case-by-case taking clinical characteristics, comorbidities, comedications, individual risk of side effects, and patients' preferences into account.
| S-EPMC8527681 | biostudies-literature
Project description:IntroductionComorbidities significantly influence the clinical course of idiopathic pulmonary fibrosis (IPF). However, their prognostic impact is not fully understood. We therefore aimed to determine the impact of comorbidities, as individual and as whole, on survival in IPF.MethodsThe database of a tertiary referral centre for interstitial lung diseases was reviewed for comorbidities, their treatments, their frequency and survival in IPF patients.Results272 patients were identified of which 12% had no, 58% 1-3 and 30% 4-7 comorbidities, mainly cardiovascular, pulmonary and oncologic comorbidities. Median survival according to the frequency of comorbidities differed significantly with 66 months for patients without comorbidities, 48 months when 1-3 comorbidities were reported and 35 months when 4-7 comorbidities were prevalent (p = 0.004). A multivariate Cox proportional hazard analyses identified other cardiac diseases and lung cancer as significant predictors of death, gastro-oesophageal reflux disease (GERD) and diastolic dysfunction had a significant positive impact on survival. A significant impact of comorbidities associated therapies on survival was not discovered. This included the use of proton pump inhibitors at baseline, which was not associated with a survival benefit (p = 0.718). We also established a predictive tool for highly prevalent comorbidities, termed IPF comorbidome which demonstrates a new relationship of IPF and comorbidities.ConclusionComorbidities are frequent in IPF patients. Some comorbidities, especially lung cancer, mainly influence survival in IPF, while others such as GERD may inherit a more favourable effect. Moreover, their cumulative incidence impacts survival.
| S-EPMC4811578 | biostudies-literature
Project description:BackgroundCurrently, there are two antifibrotics used to treat idiopathic pulmonary fibrosis (IPF): pirfenidone and nintedanib. Antifibrotics slow disease progression by reducing the annual decline of forced vital capacity (FVC), which possibly improves outcomes in IPF patients. During treatment, patients occasionally switch antifibrotic treatments. However, prognostic implication of changing antifibrotics has not yet been evaluated.MethodsThis multi-center retrospective cohort study examined 262 consecutive IPF patients who received antifibrotic therapy. Antifibrotic agents were switched in 37 patients (14.1%). The prognoses were compared between the patient cohort that switched antifibrotics (Switch-IPF) and those without (Non-Switch-IPF) using propensity-score matched analyses.ResultsThe median period between the initiation of antifibrotic therapy and the drug switch was 25.8 (12.7-35.3) months. The most common reasons for the switch were disease progression (n = 17) followed by gastrointestinal disorders (n = 12). Of the 37 patients that switched antifibrotics, only eight patients disrupted switched antifibrotics by their adverse reactions. The overall prognosis of the Switch-IPF cohort was significantly better than the Non-Switch-IPF cohort (median periods: 67.2 vs. 27.1 months, p < 0.0001). In propensity-score matched analyses that were adjusted to age, sex, FVC (%), history of acute exacerbation, and usage of long-term oxygen therapy, the Switch-IPF cohort had significantly longer survival times than the Non-Switch-IPF group (median 67.2 vs. 41.3 months, p = 0.0219). The second-line antifibrotic therapy showed similar survival probabilities than those in first-line antifibrotic therapy in multistate model analyses.ConclusionSwitching antifibrotics is feasible and may improve prognosis in patients with IPF. A further prospective study will be required to confirm clinical implication of switching the antifibrotics.
| S-EPMC8274040 | biostudies-literature
Project description:BackgroundIt is not well-known if diagnosing and treating sleep breathing disorders among individuals with idiopathic pulmonary fibrosis (IPF) improves health outcomes. We evaluated the association between receipt of laboratory-based polysomnography (which is the first step in the diagnosis and treatment of sleep breathing disorders in Ontario, Canada) and respiratory-related hospitalization and all-cause mortality among individuals with IPF.MethodsWe used a retrospective, population-based, cohort study design, analyzing health administrative data from Ontario, Canada, from 2007 to 2019. Individuals with IPF were identified using an algorithm based on health administrative codes previously developed by IPF experts. Propensity score matching was used to account for potential differences in 41 relevant covariates between individuals that underwent polysomnography (exposed) and individuals that did not undergo polysomnography (controls), in order minimize potential confounding. Respiratory-related hospitalization and all-cause mortality were evaluated up to 12 months after the index date.ResultsOut of 5044 individuals with IPF identified, 201 (4.0%) received polysomnography, and 189 (94.0%) were matched to an equal number of controls. Compared to controls, exposed individuals had significantly reduced rates of respiratory-related hospitalization (hazard ratio [HR] 0.43, 95% confidence interval [CI] 0.24-0.75), p = 0.003) and all-cause mortality (HR 0.49, 95% CI 0.30-0.80), p = 0.004). Significantly reduced rate of respiratory-related hospitalization (but not all-cause mortality) was also observed among those with > = 1 respiratory-related hospitalization (HR 0.38, 95% CI 0.15-0.99) and systemic corticosteroid receipt (HR 0.37, 95% CI 0.19-0.94) in the year prior to the index date, which reflect sicker subgroups of persons.ConclusionsUndergoing polysomnography was associated with significantly improved clinically-important health outcomes among individuals with IPF, highlighting the potential importance of incorporating this testing in IPF disease management.
| S-EPMC8170825 | biostudies-literature
Project description:BackgroundThe observation that patients with idiopathic pulmonary fibrosis (IPF) can have higher than normal expiratory flow rates at low lung volumes led to the conclusion that the airways are spared in IPF. This study aimed to re-examine the hypothesis that airways are spared in IPF using a multiresolution imaging protocol that combines multidetector CT (MDCT), with micro-CT and histology.MethodsThis was a retrospective cohort study comparing explanted lungs from patients with severe IPF treated by lung transplantation with a cohort of unused donor (control) lungs. The donor control lungs had no known lung disease, comorbidities, or structural lung injury, and were deemed appropriate for transplantation on review of the clinical files. The diagnosis of IPF in the lungs from patients was established by a multidisciplinary consensus committee according to existing guidelines, and was confirmed by video-assisted thoracic surgical biopsy or by pathological examination of the contralateral lung. The control and IPF groups were matched for age, sex, height, and bodyweight. Samples of lung tissue were compared using the multiresolution imaging approach: a cascade of clinical MDCT, micro-CT, and histological imaging. We did two experiments: in experiment 1, all the lungs were randomly sampled; in experiment 2, samples were selected from regions of minimal and established fibrosis. The patients and donors were recruited from the Katholieke Universiteit Leuven (Leuven, Belgium) and the University of Pennsylvania Hospital (Philadelphia, PA, USA). The study took place at the Katholieke Universiteit Leuven, and the University of British Columbia (Vancouver, BC, Canada).FindingsBetween Oct 5, 2009, and July 22, 2016, explanted lungs from patients with severe IPF (n=11), were compared with a cohort of unused donor (control) lungs (n=10), providing 240 samples of lung tissue for comparison using the multiresolution imaging approach. The MDCT specimen scans show that the number of visible airways located between the ninth generation (control 69 [SD 22] versus patients with IPF 105 [33], p=0·0023) and 14th generation (control 9 [6] versus patients with IPF 49 [28], p<0·0001) of airway branching are increased in patients with IPF, which we show by micro-CT is due to thickening of their walls and distortion of their lumens. The micro-CT analysis showed that compared with healthy (control) lung anatomy (mean 5·6 terminal bronchioles per mL [SD 1·6]), minimal fibrosis in IPF tissue was associated with a 57% loss of the terminal bronchioles (mean 2·4 terminal bronchioles per mL [SD 1·0]; p<0·0001), the appearance of fibroblastic foci, and infiltration of the tissue by inflammatory immune cells capable of forming lymphoid follicles. Established fibrosis in IPF tissue had a similar reduction (66%) in the number of terminal bronchioles (mean 1·9 terminal bronchioles per mL [SD 1·4]; p<0·0001) and was dominated by increased airspace size, Ashcroft fibrosis score, and volume fractions of tissue and collagen.InterpretationSmall airways disease is a feature of IPF, with significant loss of terminal bronchioles occuring within regions of minimal fibrosis. On the basis of these findings, we postulate that the small airways could become a potential therapeutic target in IPF.FundingKatholieke Universiteit Leuven, US National Institutes of Health, BC Lung Association, and Genentech.
| S-EPMC7292784 | biostudies-literature
Project description:Rationale: The diagnosis of idiopathic pulmonary fibrosis (IPF) requires exclusion of an underlying autoimmune disease, as present in interstitial lung diseases associated with connective tissue diseases (CTD-ILD). The prevalence of autoantibodies in IPF patients is currently unknown. Objectives: An unbiased assay for de novo discovery of autoantigens can help characterizing autoreactivities in IPF patients beyond clinically established autoimmune panels. Methods: We developed the proteomic Differential Antigen Capture (DAC) assay, capturing patient antibodies from plasma, followed by affinity purification coupled to mass spectrometry (AP-MS). The DAC assay quantifies the binding capacity of patient antibodies to proteins in a pooled native extract from lung explants (ILD explants n=41; donor controls n=12). Plasma antibodies from patients with IPF (n=35), CTD-ILD (n=24) and age-matched controls (n=32) were analyzed and validated in an independent cohort (IPF: n=40; CTD-ILD: n=20). Plasma antibody binding profiles were associated with clinical meta-data including diagnosis, lung function and transplant free survival. Measurements and Main Results: We identified 586 putative autoantigens in both study cohorts with a broad heterogeneity among disease entities and cohorts. On average, in IPF a mean±SD of 16±40 autoantigens and in CTD-ILD a mean±SD of 9±15 autoantigens were identified per patient. We identified 18 IPF-specific autoantigens validated in the second cohort. Interestingly, there was also a high number of shared autoantigens in IPF and CTD-ILD patients, with 17 being present in IPF and CTD-ILD of both cohorts. Presence of antibodies to Thrombospondin 1 (THBS1) and tubulin beta-1 chain (TUBB1) was associated with a significantly reduced survival in patients with IPF (p=0.002 and p=0.019, respectively). This signature was often associated with autoreactivity against talin-1 (TLN1), latent-transforming growth factor beta-binding protein 1 (LTBP1), epididymis secretory protein Li 112 (HEL-S-112), zyxin (ZYX), the LIM and senescent cell antigen-like-containing domain protein 1 (LIMS1) and caldesmon (CALD1). Conclusions: Unbiased proteomic profiling reveals that the overall prevalence of autoantibodies is similar in IPF and CTD-ILD patients and identifies novel IPF specific autoantigens associated with patient survival.
2021-02-22 | PXD024113 | Pride
Project description:Rationale: The diagnosis of idiopathic pulmonary fibrosis (IPF) requires exclusion of an underlying autoimmune disease, as present in interstitial lung diseases associated with connective tissue diseases (CTD-ILD). The prevalence of autoantibodies in IPF patients is currently unknown. Objectives: An unbiased assay for de novo discovery of autoantigens can help characterizing autoreactivities in IPF patients beyond clinically established autoimmune panels. Methods: We developed the proteomic Differential Antigen Capture (DAC) assay, capturing patient antibodies from plasma, followed by affinity purification coupled to mass spectrometry (AP-MS). The DAC assay quantifies the binding capacity of patient antibodies to proteins in a pooled native extract from lung explants (ILD explants n=41; donor controls n=12). Plasma antibodies from patients with IPF (n=35), CTD-ILD (n=24) and age-matched controls (n=32) were analyzed and validated in an independent cohort (IPF: n=40; CTD-ILD: n=20). Plasma antibody binding profiles were associated with clinical meta-data including diagnosis, lung function and transplant free survival. Measurements and Main Results: We identified 586 putative autoantigens in both study cohorts with a broad heterogeneity among disease entities and cohorts. On average, in IPF a mean±SD of 16±40 autoantigens and in CTD-ILD a mean±SD of 9±15 autoantigens were identified per patient. We identified 18 IPF-specific autoantigens validated in the second cohort. Interestingly, there was also a high number of shared autoantigens in IPF and CTD-ILD patients, with 17 being present in IPF and CTD-ILD of both cohorts. Presence of antibodies to Thrombospondin 1 (THBS1) and tubulin beta-1 chain (TUBB1) was associated with a significantly reduced survival in patients with IPF (p=0.002 and p=0.019, respectively). This signature was often associated with autoreactivity against talin-1 (TLN1), latent-transforming growth factor beta-binding protein 1 (LTBP1), epididymis secretory protein Li 112 (HEL-S-112), zyxin (ZYX), the LIM and senescent cell antigen-like-containing domain protein 1 (LIMS1) and caldesmon (CALD1). Conclusions: Unbiased proteomic profiling reveals that the overall prevalence of autoantibodies is similar in IPF and CTD-ILD patients and identifies novel IPF specific autoantigens associated with patient survival.
2021-02-22 | PXD024123 | Pride
Project description:BackgroundDelayed intubation is associated with poor prognosis in patients with respiratory failure. However, the effect of delayed intubation in patients with idiopathic pulmonary fibrosis (IPF) remains unknown. This study aimed to analyze whether timing of intubation after high-concentration oxygen therapy was associated with worse clinical outcomes in IPF patients.MethodsThis retrospective propensity score-matched study enrolled adult patients with IPF who underwent mechanical ventilation between January 2011 and July 2021. Patients were divided into early and delayed intubation groups. Delayed intubation was defined as use of high-concentration oxygen therapy for at least 48 hours before tracheal intubation. The primary outcome was intensive care unit (ICU) mortality, and a conditional logistic regression model was used to evaluate the association between timing of intubation and clinical outcomes.ResultsThe median duration of high-concentration oxygen therapy before intubation was 0.5 days in the early intubation group (n=60) and 5.1 days in the delayed intubation group (n=36). The ICU mortality rate was 56.7% and 75% in the early and delayed intubation groups, respectively, before propensity matching (P=0.075). After matching for demographic and clinical covariates, 33 matched pairs were selected. In the propensity-matched cohort, delayed intubation significantly increased the risk of ICU mortality (adjusted odds ratio, 3.99; 95% confidence interval, 1.02-15.63; P=0.046). However, in-hospital mortality did not differ significantly between the groups.ConclusionsIn patients with IPF, delayed intubation after initiation of high-concentration oxygen therapy was significantly associated with increased risk of ICU mortality compared to early intubation.
| S-EPMC9732203 | biostudies-literature