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Pfapa: Not Just for Kids
Pfapa: Not Just for Kids
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This case describes a 19-year-old woman with recurrent high fevers, sore throat, body aches, tonsillar enlargement with exudate, and tender cervical adenopathy. She had previously undergone extensive infectious and rheumatologic evaluation for similar episodes, which was unrevealing, and she had improved rapidly with corticosteroids. On this admission, broad microbiologic testing, autoimmune labs, imaging, and biopsies again failed to identify an infectious, malignant, or classic rheumatologic cause.<br /><br />Based on her recurrent pattern of fever and pharyngitis, negative workup, lack of response to antibiotics, and steroid responsiveness, she was diagnosed with PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis). Although PFAPA was originally described in children and is most common in early childhood, this report emphasizes that it can also present in young adults and should be considered in adults with unexplained recurrent febrile illness.<br /><br />The patient was treated with prednisone 60 mg with taper and colchicine 0.6 mg twice daily for prophylaxis, leading to symptomatic improvement and fever resolution. She was discharged with plans for possible tonsillectomy and confirmatory genetic testing at NIH.<br /><br />Key teaching points are that PFAPA should be considered when patients have recurrent, abrupt fever episodes with pharyngitis and cervical adenitis, minimal upper respiratory symptoms beyond throat involvement, negative infectious studies, no household spread, poor antibiotic response, and prompt improvement after a single dose of corticosteroids.
Asset Subtitle
Margaret Krasne
Meta Tag
Author List
Gigi Liu, James Son, Margaret Krasne, Timothy Kaniecki
Category
Clinical Vignettes
Concept
PFAPA Syndrome
Concept
Febrile Illness
Concept
Periodic Fever
Concept
Pharyngitis
Concept
Cervical Adenitis
Distinguished
Non-Finalist
Presenter Organization
Johns Hopkins Hospital Division of Hospital Medicine
Presenting Author
Margaret Krasne
Track
Adult
Keywords
PFAPA syndrome
periodic fever
pharyngitis
cervical adenitis
recurrent fever
tonsillar exudate
corticosteroid responsiveness
young adult
colchicine prophylaxis
tonsillectomy
PFAPA Syndrome
Febrile Illness
Periodic Fever
Pharyngitis
Cervical Adenitis
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