Heterogeneity in Unclassifiable Interstitial Lung Disease. A Systematic Review and Meta-Analysis

Guler, Sabina; Ellison, Kina; Algamdi, Mohmmed; Collard, Harold R.; Ryerson, Christopher J.

doi:10.1513/annalsats.201801-067oc

Cited by 82 publications

(70 citation statements)

References 74 publications

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“…Fibrotic iNSIP appears to have a better prognosis than other forms of progressive fibrosing ILD: considering only disease-related death, 5-year survival is approximately 75% [53]. Assessment of the mortality associated with unclassifiable IIP is hampered by the wide variability in case definition across studies, but a systematic literature review found estimates for 5-year survival in patients with unclassifiable ILD, based on four studies of 46 to 70% [54].…”

Section: Mortality In Patients With Progressing 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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Section: Mortality In Patients With Progressing Fibrosing Ildsmentioning

confidence: 99%

The natural history of progressive fibrosing interstitial lung diseases

2019

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“…Unclassifiable ILD Approximately 12% of patients with ILD cannot be classified after a diagnostic workup, and management is particularly challenging in this patient population [62]. Unclassifiable ILD is a diverse collection of patients who frequently have features resembling CTD-ILD or fibrotic HP.…”

Section: Immunosuppressive Therapiesmentioning

confidence: 99%

“…Establishing a confident ILD diagnosis is often challenging, with considerable heterogeneity in diagnoses assigned by different experienced multidisciplinary teams [47], and approximately 12% of patients remaining unclassifiable after a thorough evaluation including a surgical lung biopsy [62]. Although diagnostic certainty is often low in ILD, it is typically still feasible to characterize disease behaviour using symptoms or routine investigations such as pulmonary function tests and chest imaging.…”

Section: Is the Concept Of Pf-ild Clinically Useful?mentioning

confidence: 99%

Progression of fibrosing interstitial lung disease

2020

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Fibrotic interstitial lung diseases (ILDs) are often challenging to diagnose and classify, but an accurate diagnosis has significant implications for both treatment and prognosis. A subset of patients with fibrotic ILD experience progressive deterioration in lung function, physical performance, and quality of life. Several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Morphological similarities, common underlying pathobiologic mechanisms, and the consistently progressive worsening of these patients support the concept of a progressive fibrosing (PF)-ILD phenotype that can be applied to a variety of ILD subtypes. The conventional approach has been to use antifibrotic medications in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive medications in patients with other fibrotic ILD subtypes; however, recent clinical trials have suggested a favourable treatment response to antifibrotic therapy in a wider variety of fibrotic ILDs. This review summarizes the literature on the evaluation and management of patients with PF-ILD, and discusses questions relevant to applying recent clinicial trial findings to real-world practice.

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“…Within these specialized ILD clinics, the prevalence of unclassifiable ILD is estimated to be approximately 12%, but with substantial variability between studies likely due to heterogeneous study designs and diagnostic approaches [13]. The proportion of ILD patients who remain unclassifiable may be lower in cohorts that have undergone a multidisciplinary discussion [13], and is particularly high (up to 45%) in an elderly ILD population [25]. [26][27][28][29][30].…”

Section: Key Pointsmentioning

confidence: 99%

“…Distinguishing among ILD subtypes is frequently challenging, and accurate diagnosis often requires a multidisciplinary effort by a team of experienced ILD clinicians, chest radiologists, and lung pathologists [10 & ]. Even after a comprehensive evaluation by a group of experts, a substantial percentage of ILD patients cannot be provided with a specific diagnosis and are labeled with 'unclassifiable ILD' [11][12][13]. In this review, we summarize the evolving literature on the definition, prevalence, diagnosis, treatment, and prognosis of unclassifiable ILD, and we discuss potential approaches to its phenotyping and management.…”

Section: Introductionmentioning

confidence: 99%