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