Current classification of interstitial pulmonary diseases

Abstract

The consensus classification of idiopathic interstitial pneumonia (IIP, also known as pulmonary fibrosis) in 2002 has undergone a significant revision in 2013. What are the key points of the new consensus statement of the American Thoracic Society (ATS)/European Respiratory Society (ERS) from 2013 for the classification of pulmonary fibrosis? Evaluation of the available literature on the subject of pulmonary fibrosis in Pubmed. The interdisciplinary approach (i.e. pulmonology, radiology and pathology) for the diagnosis of lung fibrosis subtypes is highlighted. Nonspecific interstitial pneumonia (NSIP) is now a separate specific form of pulmonary fibrosis. Smoking-associated respiratory bronchiolitis with interstitial lung disease (RB-ILD) is now frequently diagnosed without a lung biopsy on the basis of clinical findings, computed tomography and bronchoalveolar lavage. The heterogeneous course of idiopathic pulmonary fibrosis (IPF) is now recognized. The term "acute exacerbation" will now be used for acute attacks (< 30 days) in patients with chronic lung fibrosis (IPF and NSIP). It has been recognized that it is necessary to provide a clinical classification algorithm for the management of IIP cases. Pleuroparenchymal fibroelastosis (PPFE) is recognized as a rare new entity of pulmonary fibrosis. The rapidly evolving field of molecular markers is promising for improving the diagnostic approach. The 2013 ATS/ERS update is a supplement to the previous classification of lung fibrosis from 2002. It outlines progress in recent years and shows potential areas for future innovations.