title: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and IgA nephropathy.
abstract: BACKGROUND
  A syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA), as well as immunoglobulin A nephropathy (IgAN), may be caused by autoimmune reactivity nephropathy.
  
  
  METHODS
  A 10-year-old boy presented with periodic fever, exudative tonsillitis, oral aphthous ulcer, and cervical lymph node inflammation. These conditions had occurred at intervals of about 2-6 weeks since the age of 3 years. Microscopic hematuria, first detected at age 8 years, worsened during episodes of PFAPA-related fever; since 10 years of age, the hematuria was accompanied by sustained proteinuria. Examination of a kidney biopsy specimen led to a diagnosis of IgAN. In the kidney specimen, fractalkine immunoreactivity and heavy macrophage infiltration were prominent. Multi-drug cocktail therapy improved the urinalysis findings, and subsequent tonsillectomy succeeded in controlling recurrences of PFAPA and IgAN. In a post-treatment renal biopsy specimen, mesangial proliferation was decreased, and fractalkine immunoreactivity was absent.
  
  
  CONCLUSIONS
  Immunologic reactions against certain antigens in local mucosa, including tonsils, may be impaired in PFAPA and IgAN, as evidenced by the suppression of both diseases in our patient by tonsillectomy. Accordingly, the concurrence of PFAPA and IgAN in our patient appeared to be a consequence of shared autoimmune mechanisms and systemic and local increases in cytokine concentrations, rather than coincidence.
authors.affiliation: Department of Pediatrics, Kinki University School of Medicine, 377-2 Ohno-higashi, Osaka-Sayama, Osaka 589-8511, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Sugimoto
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Fujita
authors.forename: Tomoki
authors.initials: T
authors.lastname: Miyazawa
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Okada
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Takemura
mesh_terms: D002648:Child; D005334:Fever / Q000150:complications / Q000276:immunology / Q000601:surgery; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000276:immunology / Q000601:surgery; D006801:Humans; D008199:Lymphadenitis / Q000150:complications* / Q000276:immunology / Q000601:surgery; D008297:Male; D010612:Pharyngitis / Q000150:complications* / Q000276:immunology / Q000601:surgery; D013281:Stomatitis, Aphthous / Q000150:complications* / Q000276:immunology / Q000601:surgery; D013577:Syndrome; D014068:Tonsillectomy