PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome and syndrome of unexplained recurrent fevers in children and adults

Elsevier

Available online 21 March 2023

The Journal of Allergy and Clinical Immunology: In PracticeAuthor links open overlay panel, , Abstract

Children 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 Words

recurrent fever

autoinflammation

PFAPA

AbbreviationsADDI

autoinflammatory disease damage index

AIDAI

autoinflammatory disease activity index

CAPS

cryopyrin associated periodic syndromes

COVID-19

coronavirus disease of 2019

FMF

familial Mediterranean fever

MKD

Mevalonate kinase deficiency

NF-ĸB

nuclear factor–ĸB

NOD

nucleotide-binding oligomerization domain

NLRC

NLR family CARD domain-containing protein

NSAIDs

non-steroidal anti-inflammatory drugs

PFAPA

periodic fever, aphthous stomatitis, pharyngitis, and adenitis

SURF

Syndrome of undifferentiated recurrent fever

TRAPS

TNF receptor-associated periodic syndrome

uSAID

undefined systemic autoinflammatory disorder

VEXAS

vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic

VUS

variant of unknown significance

© 2023 Published by Elsevier Inc. on behalf of the American Academy of Allergy, Asthma & Immunology

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