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.

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

Members of the Fleischner Society compiled a glossary of terms for thoracic imaging that replaces previous glossaries published in 1984 and 1996 for thoracic radiography and computed tomography (CT), respectively. The need to update the previous versions came from the recognition that new words have emerged, others have become obsolete, and the meaning of some terms has changed. Brief descriptions of some diseases are included, and pictorial examples (chest radiographs and CT scans) are provided for the majority of terms.

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Fleischner Society: Glossary of Terms for Thoracic Imaging

Background: In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical “gold standard” of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs.Purpose: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs.Methods: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011.Results: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis–interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific inte...

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

Idiopathic pulmonary fibrosis

Previously we described a reliable method based on immunodepletion for isolating mesenchymal stem cells (MSCs) from murine bone marrow and showed that, after intracranial transplantation, the cells migrated throughout forebrain and cerebellum and adopted neural cell fates. Here we systemically administered MSCs purified by immunodepletion from male bleomycin (BLM)-resistant BALB/c mice into female BLM-sensitive C57BL/6 recipients and quantified engraftment levels in lung by real-time PCR. Male DNA accounted for 2.21 × 10-5% of the total lung DNA in control-treated mice but was increased 23-fold (P = 0.05) in animals exposed to BLM before MSC transplantation. Fluorescence in situ hybridization revealed that engrafted male cells were localized to areas of BLM-induced injury and exhibited an epithelium-like morphology. Moreover, purification of type II epithelial cells from the lungs of transplant recipients resulted in a 3-fold enrichment of male, donor-derived cells as compared with whole lung tissue. MSC administration immediately after exposure to BLM also significantly reduced the degree of BLM-induced inflammation and collagen deposition within lung tissue. Collectively, these studies demonstrate that murine MSCs home to lung in response to injury, adopt an epithelium-like phenotype, and reduce inflammation and collagen deposition in lung tissue of mice challenged with BLM.

https://www.pnas.org/content/pnas/100/14/8407.full.pdf

Mesenchymal stem cell engraftment in lung is enhanced in response to bleomycin exposure and ameliorates its fibrotic effects.

Executive Summary Objectives Participants Evidence Validation Key Messages Introduction Rationale for a Change in the Approach to Classification of Idiopathic Interstitial Pneumonias Development of a New Classification of Idiopathic Interstitial Pneumonia Current Classification of IIP New ATS/ERS Classification Principles Guiding the Assessment of Patients with Idiopathic Interstitial Pneumonias The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis Clinical Features Radiologic Features Histologic Features IPF: Areas of Uncertainty Nonspecific Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features NSIP: Areas of Uncertainty Cryptogenic Organizing Pneumonia Clinical Features Radiologic Features Histologic Features COP: Areas of Uncertainty Acute Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features AIP: Areas of Uncertainty Respiratory Bronchiolitis-Associated Interstitial Lung Disease Clinical Features Radiologic Features Histologic Features RB-ILD: Areas of Uncertainty Desquamative Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features DIP: Areas of Uncertainty Lymphoid Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features LIP: Areas of Uncertainty References Appendix

American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias

Interstitial lung disease (ILD) is associated with rheumatoid arthritis (RA); however, the prevalence and natural history are undefined. Our aim was to determine the prevalence of ILD associated with RA using a number of sensitive techniques in patients with joint disease of less than 2-yr duration. Patients who met ARA criteria for RA were recruited from community-based and hospital rheumatologists and assessed using the following measures: clinical, lung physiology, radiology (chest X-ray, high resolution CT [HRCT]), bronchoalveolar lavage (BAL) and 99mTc-DTPA nuclear scan. Thirty-six patients (25 female and 11 male) of joint disease duration of (mean +/- SD) 13.2 +/- 8.6 mo were studied. Abnormalities consistent with ILD were found in one or more investigations in 21 of 36 (58%), which were in lung physiology in 22%, CXR in 6%, HRCT in 33%, BAL in 52%, and 99mTc-DTPA nuclear scan in 15%. Based on the results, they were categorized as having clinically significant ILD (Group 1), abnormalities compatible with ILD, but no clinically significant ILD (Group 2) and no abnormalities compatible with ILD (Group 3). Five of 36 (14%) were in Group 1, 16 of 36 (44%) in Group 2, and 15 of 36 (42%) in Group 3. The only risk factor for the presence of abnormalities compatible with ILD was male gender (p < 0.04, Student's t test). In conclusion, changes consistent with ILD in early RA are frequent. The significance of these changes is being determined in a longitudinal study.

Interstitial lung disease in recent onset rheumatoid arthritis

Cutaneous manifestations of sarcoidosis are common, but subcutaneous nodules are rare, originally described in 1904 by Darier and Roussy and thought to represent isolated skin disease. We present a 61‐year‐old male who presented with 3 months of subcutaneous nodules on the forearms and knees. Biopsy confirmed sarcoidosis. An [F‐18] fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed confluent uptake in the skin of forearms and knees, along with thighs and buttocks, mediastinal, hilar and upper abdominal lymph nodes, and multiple bones. He was well and treated with hydroxychloroquine 400 mg/day. The nodules resolved and a repeat FDG PET/CT at 5 months showed a significant decrease in the uptake at all involved sites. Although a PET scan can demonstrate extensive disease in a patient presenting with subcutaneous nodules, the literature suggests prognosis is good and treatment should start simply with the least toxic approach, such as with hydroxychloroquine therapy.

https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/rcr2.949

Subcutaneous sarcoidosis (Darier–Roussy sarcoidosis) with extensive disease on positron emission tomography: A case report and review of the literature

Fiona R. Lake

Papers

American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias

Interstitial lung disease in recent onset rheumatoid arthritis

Subcutaneous sarcoidosis (Darier–Roussy sarcoidosis) with extensive disease on positron emission tomography: A case report and review of the literature