autoinflammatory syndrome
A group of hereditary disorders characterized by recurrent episodes of systemic inflammation without high-titer autoantibodies or antigen-specific T cells.
Full Definition
Autoinflammatory syndromes are genetically determined disorders featuring recurrent episodes of systemic inflammation, fever, and organ-specific symptoms. Unlike autoimmune diseases, they lack high-titer autoantibodies or antigen-specific T cells. Examples include familial Mediterranean fever, cryopyrin-associated periodic syndromes, and TNF receptor-associated periodic syndrome. These conditions often present in childhood and require specialized treatment approaches distinct from typical autoimmune rheumatic diseases.
Usage
Usage note: Distinguish from 'autoimmune' when editing pediatric rheumatology content.
In Context
- "The patient's recurrent fevers and negative autoantibody profile suggested an underlying autoinflammatory syndrome rather than juvenile lupus." — Clinical assessment note
- "Genetic testing confirmed a cryopyrin-associated autoinflammatory syndrome in this 8-year-old with periodic fever episodes." — Diagnostic report