Late-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome: A Cue for...

PFAPA is an auto-inflammatory syndrome, characterised by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis, most commonly described in children. We report two cases of late-onset PFAPA syndrome: a 24-year-old woman and a 28-year-old man, presenting to our tertiary care center in India. In both cases, the diagnosis of PFAPA was made clinically and the patients responded well to treatment with corticosteroids. Late-onset PFAPA syndrome is an emerging but under-recognised clinical entity. Its diagnosis in adults is challenging due to overlap with conditions causing recurrent fever like chronic infections as well as a general lack of awareness of its occurrence in adults. The natural history and long-term outcomes of PFAPA in adults are not yet well-established. Recognising PFAPA or similar auto-inflammatory syndromes in the list of differentials for patients presenting with recurrent fever will help to avoid the misuse of antimicrobial therapies and facilitate targeted management.