Phenotypic Clusters Predict Outcomes in a Longitudinal Interstitial Lung Disease Cohort

Adegunsoye, Ayodeji; Oldham, Justin M.; Chung, Jonathan H.; Montner, Steven M.; Lee, Cathryn; Witt, Leah J.; Stahlbaum, Danielle; Bermea, Rene S.; Chen, Lena; Hsu, Scully; Husain, Aliya N.; Noth, Imre; Vij, Rekha; Strek, Mary E.; Churpek, Matthew M.

doi:10.1016/j.chest.2017.09.026

Cited by 43 publications

(50 citation statements)

References 35 publications

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“…Previous studies have reported a wide range of figures for the prevalence of progressive disease in patients with ILDs, reflecting the different cohorts studied and the variety of criteria used to define progression. However, it is clear that a sizeable minority of patients with CTD-ILDs, chronic HP, iNSIP, sarcoidosis-ILD and unclassifiable IIP develop a progressive fibrosing phenotype characterized by decline in lung function, worsening symptoms and premature death 4,5,7,25,27,28,[32][33][34][35] . The physicians who participated in this survey estimated that patients with non-IPF progressive fibrosing ILDs die approximately 4-5 years after initial diagnosis of ILD.…”

Section: Discussionmentioning

confidence: 99%

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

Wijsenbeek

Kreuter

Olson

et al. 2019

Current Medical Research and Opinion

180

125

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Objective: Some patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) develop a progressive fibrosing phenotype. We investigated the diagnosis and management of non-IPF ILDs using data from a survey of physicians and from US insurance claims. Methods: Pulmonologists, rheumatologists and internists in France, Germany, Italy, Japan, Spain, UK and US who had managed 10 patients with non-IPF ILDs in the past year, including those with progressive fibrosing ILDs, completed an online survey. Data on US insurance and prescription claims were obtained from a repository that aggregates data on claims routed from providers or pharmacies to payers. Results: In May-June 2017, 243 pulmonologists, 203 rheumatologists and 40 internists completed an online survey. Respondents estimated that 18-32% of patients diagnosed with non-IPF ILDs develop progressive fibrosis and that time from symptom onset to death in these patients was 61-80 months. Drug treatment was given to 50-75% of patients with non-IPF progressive fibrosing ILDs. Reasons for patients not being treated included that physicians considered patients to have mild or slowly progressing disease, or did not believe that available treatments are effective or well tolerated. Corticosteroids were the preferred first-line treatment for all types of non-IPF ILD. There was considerable heterogeneity in preferences for second-and third-line treatments. US insurance claims data from 3823 patients indicated that, in 2016, 50-75% of patients with ILDs received drug treatment (mostly corticosteroids) for their ILD. Conclusions: Physicians estimate that 18-32% of patients diagnosed with non-IPF ILDs develop a progressive fibrosing phenotype and that these patients experience significant delays in the diagnosis of ILD and the detection of progressive fibrosis. Between 25% and 50% of patients with progressive fibrosing ILDs do not receive drug therapy. There is an unmet need for effective and well tolerated treatments for progressive fibrosing ILDs.

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Section: Discussionmentioning

confidence: 99%

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

Wijsenbeek

Kreuter

Olson

et al. 2019

Current Medical Research and Opinion

180

125

View full text Add to dashboard Cite

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“…Although some patients with chronic HP experience partial recovery, patients with chronic fibrosing HP may experience rapid disease progression, particularly if the inciting antigen cannot be identified and removed [32,33]. In a recent longitudinal cohort analysis, the monthly decline in FVC % predicted over 1 year in 119 patients with chronic HP was similar to that of 286 patients with IPF [34]. In patients with stage IV pulmonary sarcoidosis, both increases and decreases in FVC and DLco may be observed over time as the disease relapses and remits [35].…”

Section: Lung Function Decline In Progressive Fibrosing Ildsmentioning

confidence: 99%

The natural history of progressive fibrosing interstitial lung diseases

2019

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A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.

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“…Investigations about the role of both genetic and environmental factors have also been conducted and showed that FOXP3 polymorphisms might determine the susceptibility IIP or CTD‐ILD in Chinese Han population and African‐American race refers to a younger age at ILD diagnosis and a better survival . A new cluster analysis among adults with various chronic ILD has been conducted, and four distinct clinical phenotypes were identified, allowing a better prediction of clinical outcomes than the current ILD diagnostic criteria. Having been said that genetic and environments are considerable impacting ILD epidemiology and natural history, we must not forget the role of co‐morbidities due to their impact on worsening of patient’s survival as seen in recent works conducted in Denmark and Germany along with the role of histopathological findings on disease progression …”

Section: Interstitial Lung Diseasementioning

confidence: 99%

Contemporary Concise Review 2018: Interstitial lung disease

2019

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Phenotypic Clusters Predict Outcomes in a Longitudinal Interstitial Lung Disease Cohort

Abstract: Among adults with diverse chronic ILDs, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might better predict meaningful clinical outcomes than current ILD diagnostic criteria.

Cited by 43 publications

References 35 publications

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

The natural history of progressive fibrosing interstitial lung diseases

Contemporary Concise Review 2018: Interstitial lung disease

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