Recurrence of Symptoms Associated with Menstruation in a Patient with a History of Periodic Fevers

Background: Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is a cyclic autoinflammatory disease generally diagnosed in childhood. There have been studies suggesting a relationship between menstruation and other autoinflammatory syndromes such as familial Mediterranean fever (FMF), but not PFAPA specifically. Case: This case describes a patient with a diagnosis of PFAPA who experienced complete resolution with tonsillectomy only to have recurrence of symptoms with onset of menstruation. She experienced symptom control with initiation of oral contraceptives. Summary and Conclusion: Prior to this case report, there had been no evidence in the literature suggesting a relationship between PFAPA and menstruation despite the observed association in other autoinflammatory syndromes. Onset of menses may be a trigger in PFAPA.