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An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-Based Guidelines for Diagnosis and Management

TLDR
This document represents the current state of knowledge regarding idiopathic pulmonary fibrosis, and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course.
Abstract
This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format. For each diagnosis and treatment question, the committee graded the quality of the evidence available (high, moderate, low, or very low), and made a recommendation (yes or no, strong or weak). Recommendations were based on majority vote. It is emphasized that clinicians must spend adequate time with patients to discuss patients' values and preferences and decide on the appropriate course of action.

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Citations
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Journal ArticleDOI

An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias

TL;DR: This update is a supplement to the previous 2002 IIP classification document and outlines advances in the past decade and potential areas for future investigation.
References
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Journal ArticleDOI

Thalidomide inhibits the intractable cough of idiopathic pulmonary fibrosis.

TL;DR: Findings are presented from a prospective cohort of 11 individuals with chronic cough caused by IPF who were enrolled in an open label phase II trial of thalidomide.
Journal ArticleDOI

Videothoracoscopic lung biopsy in diffuse infiltrative lung diseases: a 5-year surgical experience

TL;DR: Videothoracoscopic lung biopsy using an endoscopic stapler is a safe and effective procedure in most cases and should be performed by trained thoracic surgeons.
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Analysis of bronchoalveolar lavage fluid in patients with chronic hepatitis C before and after treatment with interferon alpha

TL;DR: It is suggested that HCV infection is associated with increased counts of lymphocytes and neutrophils in BAL fluid and that treatment with IFN-alpha appears to alter lymphocyte surface markers.
Journal ArticleDOI

Increased surfactant protein-A levels in patients with newly diagnosed idiopathic pulmonary fibrosis.

TL;DR: The reasons for the difference between the results and previously published reports describing decreased SP-A levels in IPF is not clear and may relate to the stricter criteria for diagnosis, the absence of treatment prior to BAL, differences in the patient population, or to other methodologic differences.
Journal ArticleDOI

Idiopathic pulmonary fibrosis: emerging concepts on pharmacotherapy.

TL;DR: Unifying concepts on the pathogenesis of IPF based on understanding of host responses to tissue injury are presented, and the importance of accurately phenotyping a highly heterogeneous disease process that may require individualised and ‘combined’ therapies is emphasised.
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American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias