- 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.
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
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
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
- Adamantiades-Behçet disease
- 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