CB03.4 Idiopathic pulmonary fibrosis
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01
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.
postcoordination
Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.
synonyms
- Idiopathic pulmonary fibrosis
- IPF - [Idiopathic pulmonary fibrosis]
- fibrosing alveolitis
- fibrosing lung disease
- fibrosing pneumonitis
- idiopathic lung fibrosis
- interstitial pulmonary fibrosis
- pulmonary fibrosis NOS
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