Methods: The study cohort consisted of 156 patients seen in the PID clinic for unexplained fever from 2008 through 2012 who were not given a definitive diagnosis or were thought to have sequential self-limited illnesses, plus 20 patients seen during that time who were diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. A scripted telephone interview with consenting parents or guardians who could be reached was conducted in 2018.
Results: Attempts were made to contact all 176 families; to date, 100 interviews have been completed. Thirteen of the children initially had prolonged, 45 recurrent, and 25 periodic fever; 17 had PFAPA. The mean follow-up period was 8 years. Only 2 patients developed new diagnoses in the interval since their initial PID visit. One who was thought to have PFAPA developed genital ulcers and was diagnosed with Behcet’s disease 4 years after the PID visit. Another who was thought to have self-limited, prolonged fever was eventually diagnosed with juvenile idiopathic arthritis. None of the remaining 98 children developed serious diagnoses. However, 14 of these children reportedly have continued fevers; 9 of the children suffer from anxiety, and 4 of the remaining 5 report good general health.
Conclusion: Most children seen in PID clinic for unexplained fever who were not given a specific diagnosis remained well after their initial visit. Two were diagnosed with autoinflammatory diseases after the appearance of telltale signs and symptoms, and none were diagnosed with immunodeficiency or cancer. The children who reportedly continue having fevers but are otherwise healthy warrant further study, with particular attention to their families’ health and illness beliefs.
V. Statler, None