Should I Consider PFAPA?
Patient Presentation
A 5-year-old female came to second opinion clinic with a 1 year history of intermittent fevers. The fevers occurred every 6 weeks for 48-72 hours with documented fevers to 102-103° F. Over the year, the fevers increased in frequency to every 3-4 weeks. She had fatigue during the fevers and mild sore throat. The patient was seen for most of these episodes. The primary care physician and an otolaryngologist documented anterior cervical adenopathy of 1.0 cm or more, and ulcerations in the mouth in most of the episodes. She was taking no medication other than acetaminophen. Review of the medical records and diaries from the family showed laboratory studies for strep pharyngitis positive one time, but all other testing for streptococcal pharyngitis were negative. Complete blood counts were normal during and between episodes, except for occasional elevated platelets, or mild leukocytosis (not at the same time) and showed no cyclicity. She also sometimes had a mildly elevated C-reactive protein. Testing for histoplasmosis and other fungal diseases were negative. She had undergone a tonsillectomy and adenoidectomy approximately 2 months ago. She had no fever for 6 weeks afterwards and then had another episode.
The past medical history showed a hospitalization for respiratory syncytial virus as an infant, and 2-3 episodes of otitis media.
The family history revealed that the family was Caucasian of northern European descent. There were no genetic abnormalities, connective tissue diseases, immunodeficiencies or children that died early in life. The younger and older siblings were healthy as were the parents.
The social history showed that the family lived on a grain farm without animals. The drinking water had been tested for infectious diseases and toxins and was negative. She had missed school because of the fevers per the school policy and fatigue but made up all her work quickly and returned to school as soon as possible. She was a good student. There was no history of travel, or exposure to persons who had traveled outside the United States or were in the military. She had two outside dogs who were healthy.
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