Monogenic autoinflammatory syndromes

Description

Monogenic hereditary autoinflammatory diseases characterised by apparently unprovoked generalised inflammation in the absence of infection or high titre autoantibodies.

Exclusions from above levels

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

Child codes

4A60.0 – Familial Mediterranean fever
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

Sibling codes

4A61 – SAPHO syndrome
4A62 – Behçet disease
4A6Y – Other specified autoinflammatory disorders
4A6Z – Autoinflammatory disorders, unspecified

Foundation entity

Monogenic autoinflammatory syndromes – http://id.who.int/icd/entity/1576710885

Part of: 04: Diseases of the immune system