RECURRENT AND CYCLIC FEVERS IN A PEDIATRIC PATIENT

Abstract

Introduction Fevers are a common complaint among the pediatric population, and are frequently related to a viral or bacterial infection that resolves with conservative treatment or antibiotics. However, fevers of significance (greater than 38.4 degrees Celsius) occurring in a cyclic pattern with unusual associated symptoms are concerning for an underlying cause. Periodic fever syndromes are inherited disorders with recurrent fever and inflammation in the absence of infection or malignancy. Case Description A 6-year old previously healthy female presented for an evaluation for periodic fevers. Starting 2 years prior, she had fevers up to 39.5 degrees Celsius every 4 weeks associated with halitosis, joint pain, headaches, and vomiting. There were no reported aphthous ulcers or diagnosed pharyngitis. Prior immune and infectious workups were negative with multiple normal CBC differentials, pan-cultures, and immunoglobulins. However, inflammatory markers were elevated during the febrile periods and decreased when afebrile. The periodic fever gene sequencing panel was negative. Her fevers responded to corticosteroid doses at the onset of the episodes, but she required monthly doses for continuing cyclic fevers. However, when colchicine therapy was initiated the fevers resolved. Discussion This patient does not fit the classic description of periodic fever aphthous stomatitis pharyngitis adenitis syndrome (PFAPA) but she has abnormal symptoms associated with cyclic fevers. Although she had a negative periodic fever genetic panel, exome sequencing is pending and she continues to follow in clinic. This case is important as it deviates from the classic PFAPA diagnosis, but the patient responds appropriately to treatment with corticosteroids and colchicine.