FB51.40

ICD-11 MMS 2026-01

Primary retroperitoneal fibrosis

Description

This is the commonest form of retroperitoneal fibrosis. By definition, no associated trigger (e.g. drugs or malignancy) can be determined, although atherosclerosis of the abdominal aorta is often associated. It has been postulated that such atherosclerosis may be of aetiological significance in some cases.

Index terms

  • Primary retroperitoneal fibrosis
  • idiopathic retroperitoneal fibrosis
  • Chronic periaortitis

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
  • 22 – Injury, poisoning or certain other consequences of external causes
  • DA0E.8 – Temporomandibular joint disorders

Sibling codes

  • FB51.4Y – Other specified retroperitoneal fibrosis
  • FB51.4Z – Retroperitoneal fibrosis, unspecified

Foundation entity

Primary retroperitoneal fibrosis – http://id.who.int/icd/entity/161159769

Part of: FB51.4: Retroperitoneal fibrosis › FB51: Fibroblastic disorders › 15: Diseases of the musculoskeletal system or connective tissue