Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis

Gimenez, Andréa; Storrer, Karin Mueller; Kuranishi, Lilian T.; Soares, Maria Raquel; Ferreira, Rimarcs Gomes; Pereira, Carlos A.C.

doi:10.1136/thoraxjnl-2017-210035

Cited by 137 publications

(118 citation statements)

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“…Most of the physicians surveyed performed pulmonary function tests every 3-6 months, and HRCT scans every 6-12 months, in their patients with ILDs. A decline in FVC or an increase in fibrotic changes on HRCT in patients with ILDs reflects disease progression and is predictive of mortality [24][25][26][27][28][29] . Thus regular monitoring is important for the early detection of patients with a progressive phenotype and could inform management decisions and patient counseling.…”

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

171

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

171

125

View full text Add to dashboard Cite

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“…Lung function tests and blood gas analyses usually have limited diagnostic value, because restrictive lung capacity and abnormal gas exchange are common to all ILDs with fibrosis [21]. However, they can help determine the severity of disease and the prognosis, and occasionally refine a working diagnosis based on disease behaviour [22][23][24][25][26][27][28]. Through serial measurements, lung function tests ( particularly FVC) provide the primary means of monitoring disease progression [21].…”

Section: Diagnostic Methodsmentioning

confidence: 99%

Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

et al. 2018

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@ERSpublicationsOther chronic ILDs with a progressive-fibrosing phenotype may have a clinical course similar to IPF. Although challenging, identification of these patients is crucial, and requires a multidisciplinary approach, to ensure optimal diagnosis and management.ABSTRACT Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.

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“…Studies in patients with progressive fibrosing ILDs have identified several factors that predict mortality, but these need to be interpreted carefully given the variation in the methodology used and the retrospective nature of most of the studies. Lower FVC is an established predictor of mortality in patients with progressive fibrosing ILDs, as evidenced by numerous studies spanning IPF [55][56][57], RA-ILD [4,49], SSc-ILD [58][59][60], chronic HP [61,62] and fibrotic iNSIP [53]. The same is true of DLco [55,56,60,[63][64][65], although this is harder to assess in multi-center studies due to a lack of standardization in its measurement.…”

Section: Predictors Of Disease Progression In Patients With Fibrosingmentioning

confidence: 99%

“…The same is true of DLco [55,56,60,[63][64][65], although this is harder to assess in multi-center studies due to a lack of standardization in its measurement. A decline in FVC > 10% predicted is another well-established predictor of mortality [49,56,62,65], but smaller declines in FVC have also been shown to be associated with a worse prognosis, at least in patients with IPF [66][67][68]. Further, it is important to bear in mind that "small" annual declines in FVC may become substantial when summed over a period of years.…”

Section: Predictors Of Disease Progression In Patients With Fibrosingmentioning

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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Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis

Cited by 137 publications

References 10 publications

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

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

Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

The natural history of progressive fibrosing interstitial lung diseases

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