1530. Parents’ Description of Febrile Episodes in Children Diagnosed with PFAPA

Abstract

BackgroundPeriodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is the most common autoinflammatory condition in children. Several guidelines have been published but still, no consensus definition exists and it represents a diagnostic challenge for the clinician. As children do not always present with the classical symptoms, early consideration through a descriptive history from the parents is essential. Our aim was to summarize parental descriptions of febrile episodes in children with PFAPA and promote timely recognition and diagnosis.MethodsThis was a retrospective cohort study of children diagnosed and managed for PFAPA in a large academic health organization with review of their charts for parental descriptors and provider documentation of febrile episodes. Parental observation of aphthous stomatitis, pharyngitis, and adenitis, and other symptoms were recorded as well as description of general clinical state during an episode, its duration, and intervals between episodes.ResultsWe identified aohort of 36 children with regular episodes of fever. Classic symptoms were absent in 28%, and regularly observed in only 44%. Children were noticed to be “fatigued,” “tired looking,” “decreased energy,” “cranky,” or “irritable.” However, the two most common parental descriptors in addition to fever were “ill-appearing” and decreased oral intake (Figure 1). When reporting episodes duration most parents estimated a few days range (Figure 2). Regular intervals of 3–4 weeks were described in 22 children (61%), while the rest had either longer intervals or wider range (Figure 3). Some variation was observed in the length and recurrences of episodes, but for individual patients, these intervals were rather consistent.ConclusionRepetitive febrile episodes are the hallmark of PFAPA. Classic supporting symptoms are often absent, but decreased energy and decreased oral intake are almost universal. Recognizing a pattern of febrile illnesses along with a parental description of abrupt onset in an ill-appearing child who is asking to be held, and refuses food should facilitate timely consideration for PFAPA rather than primary focus on the four classically attributed symptoms. Disclosures All authors: No reported disclosures.