4A60.0

ICD-11 MMS 2026-01

Familial Mediterranean fever

Description

FMF is an autoinflammatory disease associated with mutations in pyrin resulting in enhanced IL1 beta production. This results in clinical attacks of inflammation in the form of fever and serositis in the form of peritoneal, pleural or synovial inflammation along with increased acute phase reactants.

Index terms

  • Familial Mediterranean fever
  • Armenian disease
  • FMF - [familial mediterranean fever]
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  • Hereditary amyloid nephropathy
  • Periodic disease
  • Riemann periodic disease
  • Siegal Cattan Mamou disease
  • familial non-neuropathic amyloidosis
  • familial recurrent polyserositis
  • paroxysmal polyserositis
  • periodic familial peritonitis
  • periodic familial polyserositis
  • periodic fever
  • periodic polyserositis
  • Familial Mediterranean fever with amyloidosis

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A60.1 – Cryopyrin-associated periodic syndromes
  • 4A60.2 – Tumour necrosis factor receptor 1 associated periodic syndrome
  • 4A60.Y – Other specified monogenic autoinflammatory syndromes
  • 4A60.Z – Autoimflammatory syndrome, unspecified

Foundation entity

Familial Mediterranean fever – http://id.who.int/icd/entity/1373335705

Part of: 4A60: Monogenic autoinflammatory syndromes › 04: Diseases of the immune system