Clinics in chest medicine
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Idiopathic pulmonary fibrosis (IPF) is a chronic progressive and ultimately fatal disease, the clinical course of which in individual patients is highly variable. Sudden deterioration of a patient's respiratory condition during a stable course is not uncommon. ⋯ In 2007 Collard and colleagues created a Consensus Perspective, which proposed consensus definition and standard diagnostic criteria. This review primarily discusses studies performed after this Consensus Perspective.
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Interstitial lung diseases (ILDs) encompass a wide range of diffuse pulmonary disorders, characterized by a variable degree of inflammatory and fibrotic changes of the alveolar wall and eventually the distal bronchiolar airspaces. ILDs may occur in isolation or in association with systemic diseases. The clinical evaluation of a patient with ILD includes a thorough medical history and detailed physical examination; obligatory diagnostic testing includes laboratory testing, chest radiography, and high-resolution computed tomography and comprehensive pulmonary function testing and blood gas analysis. To optimize the diagnostic yield, a dynamic interaction between the pulmonologist, radiologist, and pathologist is mandatory.
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Clinics in chest medicine · Mar 2012
ReviewIdiopathic pulmonary fibrosis: phenotypes and comorbidities.
Idiopathic pulmonary fibrosis (IPF) is a progressive fatal disease of the lung with an unknown etiology and limited treatment options. Three distinct phenotypes of IPF have been proposed: combined pulmonary fibrosis and emphysema, disproportionate pulmonary hypertension in IPF, and rapidly progressive IPF. Although treatment options for IPF are limited, much can be done to identify and alleviate symptoms from comorbidities, potentially improving the overall quality of life and well-being of these patients. This article describes emerging evidence to support the hypothesis that there is more than one phenotype for IPF and describes the common comorbidities seen in this disease.
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Cigarette smoke, a toxic collection of thousands of chemicals generated from combustion of tobacco, is recognized as the primary causative agent of certain diffuse interstitial and bronchiolar lung diseases. Most patients afflicted with these disorders are cigarette smokers, and smoking cessation has been shown to be capable of inducing disease remission and should occupy a pivotal role in the management of all smokers with these diffuse lung diseases. The role of pharmacotherapy with corticosteroids or other immunomodulating agents is not well established but may be considered in patients with progressive forms of smoking-related interstitial lung diseases.
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Idiopathic pulmonary fibrosis (IPF) is a deadly progressive lung disease without an effective standard treatment approach. Because of the complexity and uncertainties of IPF treatment, therapeutic decisions need to be tailored to the individual patient, after discussing the potential benefits and pitfalls. Pirfenidone has been approved for the treatment of IPF in many countries, but is not recommended as a first-choice therapy by current guidelines because of the lack of a definite efficacy. Randomized controlled trials represent a valid choice for patients with IPF, and their completion is important in improving both survival and quality of life.