Cyclical Fevers in a 4-year-old Boy with IgA Deficiency

Abstract

While immunodeficiencies, such as (Immunoglobulin A) IgA deficiency, may predispose pediatric patients to respiratory illnesses, they are also associated with autoinflammatory conditions. Distinguishing between these possible complications requires an awareness of these uncommon conditions. We report a case of a four-year-old boy with a history of IgA deficiency presenting to his primary care provider with fever of one week’s duration accompanied by abdominal, ear, and throat pain. The patient’s mother reported that he had these symptoms twice a month for the prior ten months during which he occasionally developed oral ulcers. Between episodes, the patient fully recovered without complications. Considering the patient’s history of IgA deficiency, it was originally assumed that the patient had strep throat and coincidental resolution of his symptoms after initiation of antibiotic therapy supported this diagnosis. However, due to the recurrent nature of his symptoms, a preliminary diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) was made. A referral to an otolaryngologist was made, and findings of adenopathy and tonsillitis suggestive of PFAPA were confirmed. The patient was scheduled for adenoidectomy and tonsillectomy, which were performed without complication, leading to the cessation of these episodes.

Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

“…While there have been various reports demonstrating unregulated adaptive immune reaction in PFAPA patients, data on antibody production in PFAPA patients is not consistent due to studies showing both altered and unaltered antibody levels in the affected children. 4,[24][25][26][27][28][29][30] The present study aimed to contribute to the area of research by retrospectively analyzing serum levels of IgA, IgE, IgG, and IgM in addition to that of IgG2 and IgG3 antibodies of laterconfirmed PFAPA patients, which were not previously monitored.…”

“…While some reports demonstrated IgA, IgE, IgG, and IgM production in PFAPA patients within the normal range, others observed lowered or elevated levels in PFAPA children. 4,[24][25][26][27][28][29][30] Furthermore, IgA deficiency was suggested as a clue for the diagnosis by recent case studies. 29,31 On the other hand, in two studies and a case report on antibody levels during afebrile periods, IgA, IgG, and IgM levels were within the healthy range.…”

“…4,[24][25][26][27][28][29][30] Furthermore, IgA deficiency was suggested as a clue for the diagnosis by recent case studies. 29,31 On the other hand, in two studies and a case report on antibody levels during afebrile periods, IgA, IgG, and IgM levels were within the healthy range. 4,27,32 To our knowledge, no study has monitored the serum levels of IgG subclasses.…”