The diagnosis of Sarcoidosis is not standardized but is based on three major criteria: a compatible clinical presentation, finding nonnecrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. There are no universally accepted measures to determine if each diagnostic criterion has been satisfied so recommendations have been made within these guidelines.
This guideline provides clinical recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF). Previously defined patterns of usual interstitial pneumonia (UIP) were refined to patterns of UIP, probable UIP, indeterminate for UIP, and alternate diagnosis. For patients with newly detected interstitial lung disease (ILD) who have a high-resolution computed tomography scan pattern of probable UIP, indeterminate for UIP, or an alternative diagnosis, conditional recommendations were made for performing BAL and surgical lung biopsy.
Pirfenidone is recommended as an option for treating idiopathic pulmonary fibrosis in adults only if: (1) the person has a forced vital capacity (FVC) between 50% and 80% predicted (2) the company provides pirfenidone with the discount agreed in the patient access scheme and (3) treatment is stopped if there is evidence of disease progression (an absolute decline of 10% or more in predicted FVC within any 12‑month period).
This guideline covers diagnosing and managing idiopathic pulmonary fibrosis in people aged 18 and over. It aims to improve the quality of life for people with idiopathic pulmonary fibrosis by helping healthcare professionals to diagnose the condition and provide effective symptom management.
Nintedanib is recommended as an option for treating idiopathic pulmonary fibrosis, only if: (1) the person has a forced vital capacity (FVC) between 50% and 80% of predicted, (2) the company provides nintedanib with the discount agreed in the patient access scheme and (3) treatment is stopped if disease progresses (a confirmed decline in percent predicted FVC of 10% or more) in any 12‑month period.
Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM.
Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest
an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD).This statement proposes the term “interstitial pneumonia with autoimmune features” (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.
This Clinical Practice Guideline on the Treatment of Idiopathic Pulmonary Fibrosis updates the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guidelines from 2011 with reappraisal of previously assessed treatment options and new recommendations for novel agents.
This guideline from the UK National Institute for Health and Clinical Excellence (NICE) contains recommendations on the diagnosis of idiopathic pulmonary fibrosis and delivery of care to people with idiopathic pulmonary fibrosis, from initial suspicion of the disease and referral to a consultant respiratory physician, to best supportive care and disease-modifying treatments.
UK National Institute for Health and Clinical Excellence (NICE) technology appraisal recommends Pirfenidone as an option for treating idiopathic pulmonary fibrosis when forced vital capacity (FVC) is between 50% and 80% predicted, pirfenidone is provided with the discount agreed in the patient access scheme and it should be
discontinued if there is evidence of disease progression (a decrease in FVC of 10% or more within any 12 month period).
An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias
An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy
An Official American Thoracic Society Clinical Practice Guideline: The Clinical Utility of Bronchoalveolar Lavage Cellular Analysis in Interstitial Lung Disease
Evidence-based Guidelines for the diagnosis and management of Idiopathic Pulmonary Fibrosis, containing sections on definition, epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. An official American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/ Latin American Thoracic Association Statement.
Interstitial lung disease guideline from the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society