Unclassifiable interstitial lung diseases: Clinical characteristics and survival

Hyldgaard, Charlotte; Bendstrup, Elisabeth; Wells, Athol U.; Hilberg, Ole

doi:10.1111/resp.12931

Cited by 63 publications

(64 citation statements)

References 15 publications

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“…Hyldgaard et al . describe the clinical characteristics of a cohort of 105 (24% of the total ILD referrals) patients with unclassifiable ILD, with intermediate survival rates between IPF and non‐IPF ILD, in keeping with previous reports . Interestingly, the gender age physiology (GAP) severity score and the disease behaviour classification were both independently predictive of outcome, an observation of practical utility in this patient group, where uncertainty can paralyse management.…”

Section: Interstitial Lung Diseasesupporting

confidence: 79%

Year in review 2017: Interstitial lung disease, pulmonary vascular disease and sleep

et al. 2018

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Section: Interstitial Lung Diseasesupporting

confidence: 79%

Year in review 2017: Interstitial lung disease, pulmonary vascular disease and sleep

et al. 2018

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“…Unclassifiable IIP IIPs may remain unclassifiable due to inadequate, nonspecific or conflicting clinical, radiological or histopathological findings, or because patients are unwilling or unable to undergo lung biopsy [16,17,42,43]. Unclassifiable IIP was first defined in international guidelines in 2013, meaning the disease has been largely unrecognised and further investigation is needed for improved understanding [16,44].…”

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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“…Composite scoring systems have been developed to predict mortality in patients with progressive fibrosing ILDs. One of the most widely used is the GAP (gender, age, physiology) model, which was developed to predict mortality in patients with IPF based on gender, age, FVC % predicted and DLco % predicted [69], and has since been shown to predict mortality in patients with RA-ILD [70], SSc-ILD [71], unclassifiable ILD [72] and a mixed cohort [73]. Composite scoring systems have also been developed specifically for prediction of progression of SSc-ILD [74,75].…”

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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Unclassifiable interstitial lung diseases: Clinical characteristics and survival

Abstract: The DBC approach showed strong prognostic value in unclassifiable ILD. The DBC and the ILD-GAP were complementary predictors of outcome in unclassifiable ILD.

Cited by 63 publications

References 15 publications

Year in review 2017: Interstitial lung disease, pulmonary vascular disease and sleep

Year in review 2017: Interstitial lung disease, pulmonary vascular disease and sleep

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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