Management of Idiopathic Pulmonary Fibrosis. 2021

Margaret L Salisbury, and Marlies S Wijsenbeek
Department of Medicine, Division of Allergy, Pulmonary and Critical Care, Vanderbilt University Medical Center, 1161 21st Avenue South, T-1209A Medical Center North, Nashville, TN 37232, USA. Electronic address: Margaret.Salisbury@vumc.org.

Progress in the past 2 decades has led to widespread use of 2 medications to slow loss of lung function in patients with pulmonary fibrosis. Treatment of individual patients with currently available pharmacotherapies can be limited by side effects, and neither drug has a consistent effect on patient symptoms or function. Several promising new pharmacotherapies are under development. Comprehensive management of pulmonary fibrosis hinges on shared decision making. Patient and caregiver education, and early identification and management of symptoms and comorbidities, can help improve quality of life.

UI MeSH Term Description Entries
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D015897 Comorbidity The presence of co-existing or additional diseases with reference to an initial diagnosis or with reference to the index condition that is the subject of study. Comorbidity may affect the ability of affected individuals to function and also their survival; it may be used as a prognostic indicator for length of hospital stay, cost factors, and outcome or survival.
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

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