Available online 21 March 2023
Author links open overlay panel, , AbstractChildren and adults with autoinflammatory disorders, who often experience recurrent fevers, rashes, cold-induced symptoms, conjunctivitis, lymphadenopathy, recurrent infections, aphthous stomatitis and abnormal blood cell counts, may present to the allergist/immunologist because their symptoms mimic allergies and disorders of immunity. In recent years, there has been increased recognition of non-monogenic autoinflammatory disorders, including periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and syndrome of undifferentiated recurrent fevers (SURF). For many clinical practitioners, the natural history, diagnostic criteria, differential diagnoses, and preferred therapies remain a challenge due to the presumed rarity of the patients and evolving field of autoinflammation. Here, we aim to provide a practical framework for the clinical allergist/immunologist to evaluate and treat this patient population.
Key Wordsrecurrent fever
autoinflammation
PFAPA
AbbreviationsADDIautoinflammatory disease damage index
AIDAIautoinflammatory disease activity index
CAPScryopyrin associated periodic syndromes
COVID-19coronavirus disease of 2019
FMFfamilial Mediterranean fever
MKDMevalonate kinase deficiency
NF-ĸBnuclear factor–ĸB
NODnucleotide-binding oligomerization domain
NLRCNLR family CARD domain-containing protein
NSAIDsnon-steroidal anti-inflammatory drugs
PFAPAperiodic fever, aphthous stomatitis, pharyngitis, and adenitis
SURFSyndrome of undifferentiated recurrent fever
TRAPSTNF receptor-associated periodic syndrome
uSAIDundefined systemic autoinflammatory disorder
VEXASvacuoles, E1 enzyme, X-linked, autoinflammatory, somatic
VUSvariant of unknown significance
© 2023 Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology
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