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...

/pdf/an-official-american-thoracic-society-european-respiratory-35zyfky253.pdf

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

Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some patients live much longer. Respiratory failure resulting from disease progression is the most frequent cause of death. To date we have limited information as to predictors of mortality in patients with IPF, and research in this area has failed to yield prediction models that can be reliably used in clinical practice to predict individual risk of mortality. The goal of this concise clinical review is to examine and summarize the current data on the clinical course, individual predictors of survival, and proposed clinical prediction models in IPF. Finally, we will discuss challenges and future directions related to predicting survival in IPF.

Clinical course and prediction of survival in idiopathic pulmonary fibrosis.

https://www.thelancet.com/article/S2213260020301685/pdf

Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society

The widespread use of high-resolution computed tomography in clinical and research settings has increased the detection of interstitial lung abnormalities (ILA) in asymptomatic and undiagnosed individuals. We reported that in smokers, ILA were present in about 1 of every 12 high-resolution computed tomographic scans; however, the long-term significance of these subclinical changes remains unclear. Studies in families affected with pulmonary fibrosis, smokers with chronic obstructive pulmonary disease, and patients with inflammatory lung disease have shown that asymptomatic and undiagnosed individuals with ILA have reductions in lung volume, functional limitations, increased pulmonary symptoms, histopathologic changes, and molecular profiles similar to those observed in patients with clinically significant interstitial lung disease (ILD). These findings suggest that, in select at-risk populations, ILA may represent early stages of pulmonary fibrosis or subclinical ILD. The growing interest surrounding this topic is motivated by our poor understanding of the inciting events and natural history of ILD, coupled with a lack of effective therapies. In this perspective, we outline past and current research focused on validating radiologic, physiological, and molecular methods to detect subclinical ILD. We discuss the limitations of the available cross-sectional studies and the need for future longitudinal studies to determine the prognostic and therapeutic implications of subclinical ILD in populations at risk of developing clinically significant ILD.

Subclinical interstitial lung disease: why you should care.

Non-neoplastic Disorders of the Lower Respiratory Tract

MR features of a case of chronic expanding hematoma.

Background and objective:  The clinical features and acute component of the pathological findings in patients with acute exacerbation of IPF are similar to those of acute interstitial pneumonia (AIP). A growing body of research suggests that acute exacerbation of subclinical IPF is similar to AIP. This study tested the hypothesis that subpleural fibrosis (resembling localized usual interstitial pneumonia) in patients with diffuse alveolar damage can mimic patients with AIP alone.



Methods:  The clinical, radiological and histopathological characteristics of patients with AIP with or without subpleural fibrosis, as identified at post-mortem examination, were assessed.



Results:  Fourteen patients with AIP met the inclusion criteria. These comprised nine men and five women ranging in age from 65 to 90 years. The mean duration from onset of symptoms to admission was 12.3 days, and from admission to death was 50.5 days. All patients presented with severe respiratory failure with bilateral pulmonary infiltrates on CXR. CT scans revealed bilateral ground glass opacity (100.0%), air space consolidation (69.2%) and no honeycombing. In seven patients (50.0%) histological examination showed subpleural fibrosis. There were no differences in the clinicopathological or CT scan findings between patients with AIP only compared with those with AIP and subpleural fibrosis.



Conclusions:  Subpleural fibrosis resembling localized usual interstitial pneumonia is often present in patients diagnosed as having AIP. These patients were not distinguishable from those with AIP only, based on the clinicopathological and radiological findings.

Clinically occult subpleural fibrosis and acute interstitial pneumonia a precursor to idiopathic pulmonary fibrosis

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MR features of a case of chronic expanding hematoma.

Clinically occult subpleural fibrosis and acute interstitial pneumonia a precursor to idiopathic pulmonary fibrosis