Idiopathic pulmonary fibrosis

Definition

Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia (pneumonitis) of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of UIP (Usual Interstitial Pneumonia). The definition of IPF requires the exclusion of other forms of interstitial pneumonia (pneumonitis) including other idiopathic interstitial pneumonias (pneumonitis) and Interstitial Lung Disease (ILD) associated with environmental exposure, medication, or systemic disease.

Also indexed as

Nearby in Idiopathic interstitial pneumonitis

• CB03.0Acute interstitial pneumonitis
• CB03.1Combined pulmonary fibrosis and emphysema syndrome
• CB03.2Cryptogenic organizing pneumonitis
• CB03.3Desquamative interstitial pneumonitis
• CB03.5Lymphoid interstitial pneumonia
• CB03.6Respiratory bronchiolitis - interstitial lung disease
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified