Idiopathic pulmonary fibrosis

Description

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.

Index terms

Idiopathic pulmonary fibrosis
IPF - [Idiopathic pulmonary fibrosis]
fibrosing alveolitis

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fibrosing lung disease
fibrosing pneumonitis
idiopathic lung fibrosis
interstitial pulmonary fibrosis
pulmonary fibrosis NOS

Exclusions from above levels

01 – Certain infectious or parasitic diseases
05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth and the puerperium

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19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations and chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

CB03.0 – Acute interstitial pneumonitis
CB03.1 – Combined pulmonary fibrosis and emphysema syndrome
CB03.2 – Cryptogenic organizing pneumonitis
CB03.3 – Desquamative interstitial pneumonitis
CB03.5 – Lymphoid interstitial pneumonia
CB03.6 – Respiratory bronchiolitis - interstitial lung disease
CB03.Y – Other specified idiopathic interstitial pneumonitis
CB03.Z – Idiopathic interstitial pneumonitis, unspecified

Foundation entity

Idiopathic pulmonary fibrosis – http://id.who.int/icd/entity/1074069640

Allowed postcoordination axes

Part of: CB03: Idiopathic interstitial pneumonitis › 12: Diseases of the respiratory system