Idiopathic pulmonary fibrosis classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Idiopathic pulmonary fibrosis is a disease among a large group of the interstitial lung diseases and particularly a subtype of the idiopathic interstitial pneumonia. The major group Acute Interstitial Pneumonia (AIP), Cryptogenic Organizing Pneumonia (COP), Respiratory Bronchiolitis–Interstitial Lung Disease (RB-ILD), and Desquamative Interstitial Pneumonia (DIP)

Classification

• Idiopathic pulmonary fibrosis is a disease among a large group of the interstitial lung diseases and particularly a subtype of the idipathic interstitial pneumonia. The major forms of the idiopathic interstitial pneumonia include
• The idiopathic interstitial pneumonias are classified by the American Thoracic Society/European Respiratory Society (ATS/ERS) into three main groups:
  • Major idiopathic interstitial pneumonia
  • Rare idiopathic interstitial pneumonia
  • Unclassificable idiopathic interstitial pneumonia
• The idiopathic interstitial fibrosis is the chronic form of the idiopathic interstitial pneumonia. Other forms of the idiopathic interstitial pneumonia include the following:
  • Acute or subacute: Acute Interstitial Pneumonia (AIP) and Cryptogenic Organizing Pneumonia (COP)
  • Chronic: Idiopathic Pulmonary Fibrosis (IPF)
  • Smoking related: Respiratory Bronchiolitis–Interstitial Lung Disease (RB-ILD) and Desquamative Interstitial Pneumonia (DIP)