CB03

ICD-11 MMS 2026-01

Idiopathic interstitial pneumonitis

Description

The idiopathic interstitial pneumonias (pneumonitises) are a subset of diffuse interstitial lung diseases of unknown etiology characterised by expansion of the interstitial compartment (i.e. that portion of the lung parenchyma sandwiched between the epithelial and endothelial basement membranes) with an infiltrate of inflammatory cells. The inflammatory infiltrate is sometimes accompanied by fibrosis, either in the form of abnormal collagen deposition or proliferation of fibroblasts capable of collagen synthesis.

Inclusions

  • Idiopathic interstitial pneumonia

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

Child codes

  • CB03.0 – Acute interstitial pneumonitis
  • CB03.1 – Combined pulmonary fibrosis and emphysema syndrome
  • CB03.2 – Cryptogenic organizing pneumonitis
  • CB03.3 – Desquamative interstitial pneumonitis
  • CB03.4 – Idiopathic pulmonary fibrosis
  • 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

Sibling codes

  • CB00 – Acute respiratory distress syndrome
  • CB01 – Pulmonary oedema
  • CB02 – Pulmonary eosinophilia
  • CB04 – Primary interstitial lung diseases specific to infancy or childhood
  • CB05 – Interstitial lung diseases associated with systemic diseases
  • CB06 – Pulmonary alveolar microlithiasis
  • CB07 – Lymphangioleiomyomatosis
  • CB0Y – Other specified respiratory diseases principally affecting the lung interstitium
  • CB0Z – Respiratory diseases principally affecting the lung interstitium, unspecified

Foundation entity

Idiopathic interstitial pneumonitis – http://id.who.int/icd/entity/1297512293

Allowed postcoordination axes

Part of: 12: Diseases of the respiratory system