SHM Converge Scientific Abstract ePoster Gallery-A Unique Case of Pfapa Syndrome Recurrence in Adulthood

Pdf Summary

This case report describes a rare recurrence of PFAPA syndrome in adulthood. PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) is an autoinflammatory periodic fever syndrome usually seen in children, characterized by recurrent fevers with sore throat, mouth ulcers, and cervical lymphadenopathy, along with symptom-free intervals and normal growth.<br /><br />The patient was a 21-year-old woman with a one-year history of intermittent fevers lasting 4–5 days, occurring about monthly, and associated with sore throat, enlarged cervical lymph nodes, and oral ulcers. She had experienced similar episodes in childhood that resolved after tonsillectomy. Because adult PFAPA is uncommon and lacks universally accepted diagnostic criteria, her presentation initially prompted an extensive evaluation for infection, autoimmune disease, and malignancy. Workup, including cultures, imaging, inflammatory markers, serologic testing, and lymph node biopsy, was unrevealing.<br /><br />She was ultimately diagnosed clinically with PFAPA syndrome. Her symptoms improved with colchicine 0.6 mg every other day, and prednisone 30 mg was effective as abortive therapy during flares.<br /><br />The report emphasizes that PFAPA should be considered in adults with cyclical fever of unknown origin, especially when episodes include aphthous ulcers, pharyngitis, and adenitis. It also highlights the need for further research into triggers and risk factors for recurrence, which could lead to earlier diagnosis, avoid unnecessary testing, and improve patient quality of life.

Asset Subtitle

Patrick M. Kukulich

Meta Tag

Author List Marie Dimattia, Patrick M. Kukulich

Category Clinical Vignettes

Concept PFAPA Syndrome

Concept Recurrent Fever

Concept Autoinflammatory Periodic Fever Syndrome

Concept Aphthous Stomatitis

Concept Pharyngitis

Distinguished Non-Finalist

Presenter Organization Thomas Jefferson University Hospitals

Presenting Author Patrick M. Kukulich

Track Adult

Keywords

PFAPA syndrome

adult recurrence

periodic fever

aphthous stomatitis

pharyngitis

cervical adenitis

cyclical fever

tonsillectomy

colchicine

prednisone

PFAPA Syndrome

Recurrent Fever

Autoinflammatory Periodic Fever Syndrome

Aphthous Stomatitis

Pharyngitis