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Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome

  • Kathryn M. EdwardsEmail author
  • Michael Hofer
Chapter

Abstract

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome that usually starts in early childhood. Episodes occur every 4–6 weeks and spontaneously resolve in 3–6 days. During episodes, children have elevated acute phase reactants that return to normal between episodes. Children with PFAPA grow and develop normally, are not more susceptible to infections, and exhibit no long-term sequelae. Since its initial description in 1987, numerous cases of PFAPA have been reported throughout the world and understanding of the syndrome has greatly increased. The epidemiology, theories on causation, clinical manifestations, and therapeutic options for PFAPA will the discussed in this chapter.

Keywords

Periodic fever Aphthous stomatitis Pharyngitis Cervical adenitis (PFAPA) Periodic fever Autoinflammation 

Abbreviations

CAPS

Cryopyrin-associated periodic syndrome

CARD

Caspase activation and recruiting domains

CRP

C-reactive protein

ESR

Erythrocyte sedimentation rate

FMF

Familial Mediterranean fever

HIDS

Hyperimmunoglobulinemia D syndrome and periodic fever

Ig

Immunoglobulin

IL

Interleukin

IL-1RA

Interleukin-1 receptor antagonist

INF

Interferon

IP10

Interferon γ-induced protein

LPS

Lipopolysaccharide

MKD

Mevalonate kinase deficiency

NLRP

Nucleotide-binding oligomerization domain, leucine rich repeat with pyrin domain

PCR

Polymerase chain reaction

PD-1

Programmed cell death protein 1

PFAPA

Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis

TNF

Tumor necrosis factor

TRAPS

TNF receptor-associated periodic syndrome

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Division of Infectious Diseases, Department of PediatricsVanderbilt University School of Medicine, Monroe Carell, Jr. Children’s Hospital at VanderbiltNashvilleUSA
  2. 2.Unit of Pediatric Immunology and Rheumatology of Western Switzerland, CHUVUniversity of LausanneLausanneSwitzerland
  3. 3.University Hospital of GenevaGenevaSwitzerland

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