Periodic Fever and Autoinflammatory Syndromes

Periodic Fever and Autoinflammatory Syndromes

• Autoinflammatory syndromes typically present as recurrent episodes of inflammation without evidence of infection, autoantibodies, or antigen-specific T cells.

• Autoinflammatory syndromes are characterized by episodes of fever, rash, arthritis, peritonitis, eye inflammation, and elevated acute-phase reactants in various combinations that normalize between flares; there is a great deal of symptom overlap amongst different autoinflammatory syndromes.

• Many of the autoinflammatory syndromes are monogenetic diseases with mutations altering the function of the inflammatory cascade.

• In the cryopyrin-associated periodic syndromes (CAPS), nucleotide-binding oligomerization domain, leucine-rich repeat, and pyrin domain-containing 3 (NLRP3) mutations cause dysfunction of the inflammasome leading to abnormal interleukin 1β (IL-1β) production.

• Amyloidosis (AA) is a rare and late complication of some autoinflammatory syndromes and can lead to renal failure in untreated patients.

• Autoinflammatory syndromes are rare; they are more likely in patients who have younger age of onset (infancy or childhood), a familial pattern of illness, severe manifestations, and clinical response to medication.

• IL-1β inhibitors are an effective therapy for many, but not all, of these diseases.

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