4A60.2

ICD-11 MMS 2026-01

Tumour necrosis factor receptor 1 associated periodic syndrome

Description

TRAPS is an autoinflammatory disease associated with heterozygous mutations in the gene coding for tumour necrosis factor (TNF) receptor 1 (TNFR1). 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

  • Tumour necrosis factor receptor 1 associated periodic syndrome
  • Autosomal dominant periodic fever
  • Familial Hibernian fever
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  • TNF - [tumour necrosis factor] receptor 1 associated periodic syndrome
  • TRAPS - [tumour necrosis factor receptor 1 associated periodic syndrome]
  • Tumour necrosis factor receptor 1 associated periodic syndrome with amyloidosis

Exclusions from above levels

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

Sibling codes

  • 4A60.0 – Familial Mediterranean fever
  • 4A60.1 – Cryopyrin-associated periodic syndromes
  • 4A60.Y – Other specified monogenic autoinflammatory syndromes
  • 4A60.Z – Autoimflammatory syndrome, unspecified

Foundation entity

Tumour necrosis factor receptor 1 associated periodic syndrome – http://id.who.int/icd/entity/1869883509

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