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4A60.1 Cryopyrin-associated periodic syndromes

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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

synonyms

• Cryopyrin-associated periodic syndromes
• CAPS - [Cryopyrin-associated periodic syndromes]
• Cryopyrinopathies
• Chronic infantile neurological, cutaneous and articular syndrome
• Infantile-onset multisystem inflammatory disease
• Neonatal-onset multisystem inflammatory disease
• Prieur-Griscelli syndrome
• NOMID - [Neonatal-onset multisystem inflammatory disease]
• IOMID - [Infantile-onset multisystem inflammatory disease]
• CINCA - [Chronic infantile neurological, cutaneous and articular] syndrome
• Chronic infantile neurological cutaneous and articular syndrome with CNS disease
• Familial cold autoinflammatory syndrome
• Familial cold urticaria
• Muckle-Wells syndrome
• Urticaria - deafness - amyloidosis
• Muckle Wells Syndrome with amyloidosis
• Muckle Wells Syndrome with neurosensorial hearing loss

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