1146. Immune Function of Tonsillar Tissue and Blood in Patients with Periodic Fever, Adenitis, Pharyngitis and Aphthous Ulcers (PFAPA)
Session: Poster Abstract Session: Innate and Adaptive Immunity to Infections
Saturday, October 22, 2011
Room: Poster Hall B1
  • IDSA 2011 2-1.pdf (861.0 kB)


    Background: Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) is a common causes of recurrent fever in childhood. The cause of PFAPA remains unknown.  Similar to other periodic fever syndromes, increased production of IgD and altered immune cell function may play a role in the inflammatory process of PFAPA.

    Methods: Adenoid, tonsils and peripheral blood of four PFAPA patients were obtained at the time of adenoidectomy and/or tonsillectomy. Samples from four age-matched patients with obstructive sleep apnea undergoing tonsillectomy served as controls. Frozen adenoid and/or tonsil sections were analyzed by immunofluorescence. Cells in peripheral blood were analyzed by flow cytometry in two PFAPA patients. Serum IgD levels were measured by ELISA.

                                Patient Characteristics


    Patient ID

     Age at Onset

    Age at Tonsillectomy


    Serum IgD (IU/mL)

    Respond to Corticosteroid


    24 mo

    30 mo





    25 mo

    66 mo





    24 mo

    31 mo





    48  mo

    65 mo




    Results: Increased infiltration of elastase+ neutrophils to the perifollicular regions of adenoid and tonsils was observed in all PFAPA patients. In all PFAPA patients, follicular hyperplasia accompanied by extensive proliferation of cells in adenoid and tonsil follicles was observed, suggesting exaggerated germinal center reactions. There was a significantly lower percentage (p = 0.012) of CD19+ B cells in circulating mononuclear cells in PFAPA patients (10.79±0.41) as compared to controls (17.57±1.47). There was increased expression of mucosal homing receptors integrin-α4, integrin-β7, CCR10 and the activation marker CD38 on circulating IgMIgD class switched B cells in PFAPA patients. Increased expression of MAdCAM-1 and integrin-β7 was found on circulating basophils, and decreased circulating basophil percentage in PFAPA patients(0.2±0.1) as compared to controls (0.95±0.27).

    Conclusion: There are altered local and systemic immune responses in PFAPA. Aberrant upregulation of mucosal homing receptors on circulating B cells and basophils may promote their infiltration into mucosal tissues, which may explain the local inflammatory reactions that underlie the pathology of PFAPA.

    Subject Category: P. Pediatric and perinatal infections

    Patricia DeLaMora, MD1, Kang Chen, PhD2, Max April, MD3 and Andrea Cerutti, MD2, (1)Pediatrics, Weill Cornell Medical College, New York, NY, (2)Medicine, Mount Sinai School of Medicine, New York, NY, (3)Otorhinolaryngology, Weill Cornell Medical Center, New York, NY


    P. DeLaMora, None

    K. Chen, None

    M. April, None

    A. Cerutti, None

    Findings in the abstracts are embargoed until 12:01 a.m. EST Thursday, Oct. 20 with the exception of research findings presented at IDSA press conferences.