Autoinflammatory Disorders – Definitions & ICD 11 Codes

Coded Elsewhere:

• Schnitzler syndrome (EB03)

Monogenic autoinflammatory syndromes

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

ICD 11 Code For Monogenic autoinflammatory syndromes

  4A60  Monogenic autoinflammatory syndromes

4A60.0 Familial Mediterranean fever

Definition of Familial Mediterranean fever: 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.

4A60.1 Cryopyrin-associated periodic syndromes

Definition of Cryopyrin-associated periodic syndromes: CAPS is an autoinflammatory disease associated with gain of function changes in the cryopyrin protein, resulting in inflammasome activation and enhanced IL1 beta production. This results in clinical signs and symptoms of inflammation in the form of rash, fever, joint and eye symptoms with increased acute phase reactants.

Inclusions:

• Cryopyrinopathies

4A60.2 Tumour necrosis factor receptor 1 associated periodic syndrome

Definition of Tumour necrosis factor receptor 1 associated periodic syndrome: 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.

4A60.Y Other specified monogenic autoinflammatory syndromes

4A60.Z Autoimflammatory syndrome unspecified

SAPHO syndrome

Definition of SAPHO syndrome: SAPHO syndrome is characterised by a constellation of symptoms and signs including synovitis, acne conglobata or fulminans, palmoplantar pustulosis, hyperostosis and osteitis. Its aetiology is poorly understood.

ICD 11 Code For SAPHO syndrome

  4A61  SAPHO syndrome

Behçet disease

Definition of Behçet disease: Behçet disease is a disease of incompletely understood aetiopathogenesis characterised by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. Small vessel vasculitis, thrombotic vasculopathy, arteritis and arterial aneurysms may occur. It has a high prevalence from the Eastern Mediterranean across Central Asia to China and Japan.

ICD 11 Code For Behçet disease

  4A62  Behçet disease

Inclusions:

• Adamantiades-Behçet disease

Coded Elsewhere:

• Transient neonatal Behçet disease (KA07.Y)

Other specified autoinflammatory disorders

ICD 11 Code For Other specified autoinflammatory disorders

  4A6Y  Other specified autoinflammatory disorders

Autoinflammatory disorders unspecified

ICD 11 Code For Autoinflammatory disorders unspecified

  4A6Z  Autoinflammatory disorders unspecified