periodic fever, aphthous stomatitis, pharyngitis and adenitis

ICD-10: E85.0ORPHA: 85450

Overview

PFAPA (Periodic fever - aphthous stomatitis- pharyngitis - adenopathy) syndrome is an auto inflammatory syndrome characterized by recurrent febrile episodes associated with aphthous stomatitis, pharyngitis and cervical adenitis

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with periodic fever, aphthous stomatitis, pharyngitis and adenitis, sourced from HPO and Orphanet clinical annotations.

ProteinuriaNephropathyRenal interstitial amyloid depositsRenal insufficiencyHypertensionTubulointerstitial nephritisElevated circulating creatinine concentrationAbnormality of the gastrointestinal tractDecreased glomerular filtration rateAbnormal urinary electrolyte concentrationNocturiaRenal tubular atrophyNephrotic syndromePetechiaePurpuraDecreased liver functionHepatosplenomegalyCongestive heart failureWeight lossAnemiaVomitingDiarrheaNauseaGastroesophageal refluxAbdominal painGastrointestinal hemorrhageHepatomegalyLymphadenopathyAbnormal lymph node morphologyMyopathyDecreased HDL cholesterol concentrationElevated erythrocyte sedimentation rateIntestinal obstructionTubulointerstitial fibrosisPeripheral neuropathyIntestinal perforationDecreased circulating apolipoprotein A-I concentrationDyspepsiaAbnormal testis morphologyHypogonadismOligozoospermiaMale infertilityPrimary testicular failure

Classification & Codes

ICD-10 Code

E85.0

Orphanet Code

ORPHA:85450
periodic fever, aphthous stomatitis, pharyngitis and adenitis
ICD-10E85.0
OrphanetORPHA:85450
Treatments0 drug(s)
Symptoms on record43 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO