Biomaterial Database

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. 2019

Marlies Wijsenbeek, and Michael Kreuter, and Amy Olson, and Aryeh Fischer, and Elisabeth Bendstrup, and Christopher D Wells, and Christopher P Denton, and Baher Mounir, and Leila Zouad-Lejour, and Manuel Quaresma, and Vincent Cottin

Department of Respiratory Medicine, Erasmus MC, University Medical Centre , Rotterdam , The Netherlands.

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.

UI	MeSH Term	Description	Entries
D010641	Phenotype	The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.	Phenotypes
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D017563	Lung Diseases, Interstitial	A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.	Diffuse Parenchymal Lung Disease,Diffuse Parenchymal Lung Diseases,Interstitial Lung Disease,Interstitial Lung Diseases,Pneumonia, Interstitial,Pneumonitis, Interstitial,Interstitial Pneumonia,Interstitial Pneumonias,Interstitial Pneumonitides,Interstitial Pneumonitis,Lung Disease, Interstitial,Pneumonias, Interstitial,Pneumonitides, Interstitial
D054990	Idiopathic Pulmonary Fibrosis	A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.	Cryptogenic Fibrosing Alveolitis,Familial Idiopathic Pulmonary Fibrosis,Fibrocystic Pulmonary Dysplasia,Fibrosing Alveolitis, Cryptogenic,Idiopathic Fibrosing Alveolitis, Chronic Form,Idiopathic Pulmonary Fibrosis, Familial,Interstitial Pneumonitis, Usual,Pulmonary Fibrosis, Idiopathic,Usual Interstitial Pneumonia,Cryptogenic Fibrosing Alveolitides,Dysplasia, Fibrocystic Pulmonary,Fibrocystic Pulmonary Dysplasias,Fibrosing Alveolitides, Cryptogenic,Idiopathic Pulmonary Fibroses,Interstitial Pneumonia, Usual,Pneumonitides, Usual Interstitial,Pneumonitis, Usual Interstitial,Pulmonary Dysplasia, Fibrocystic,Pulmonary Fibroses, Idiopathic,Usual Interstitial Pneumonias,Usual Interstitial Pneumonitides,Usual Interstitial Pneumonitis
D018450	Disease Progression	The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.	Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease