CB22.0

ICD-11 MMS 2026-01

Fibrosing mediastinitis

Description

Fibrosing mediastinitis, also known as sclerosing mediastinitis or mediastinal fibrosis, is a disorder characterised by an excessive fibrotic reaction in the mediastinum. It can result in compromise of airways, great vessels, and other mediastinal structures, with morbidity directly related to the location and extent of fibrosis. The commonest cause is histoplasmosis, of which it is a rare late complication, but it may also occur in association with other infections and with systemic autoimmune disorders such as Behçet disease, granulomatosis with polyangiitis and retroperitoneal fibrosis.

Index terms

  • Fibrosing mediastinitis
  • idiopathic mediastinal fibrosis
  • mediastinal fibrosis
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  • Histoplasmosis-related fibrosing mediastinitis
  • Histoplasmosis-related mediastinal fibrosis mediastinitis
  • Fibrosing mediastinitis in association with systemic autoimmune disease
  • Idiopathic hilar fibrosis

Exclusions from above levels

  • CA43.3 – Abscess of mediastinum
  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 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

  • CB22.Y – Other specified diseases of mediastinum, not elsewhere classified
  • CB22.Z – Disease of mediastinum, unspecified

Foundation entity

Fibrosing mediastinitis – http://id.who.int/icd/entity/123840075

Part of: CB22: Diseases of mediastinum, not elsewhere classified › 12: Diseases of the respiratory system