- PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND ADENITIS (PFAPA).

PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND ADENITIS (PFAPA)

PFAPA is the commonest periodic fever syndrome in childhood.

It usually presents between the ages of 2 and 5 yr.

It is unlikely to be due to a single gene .

Diagnosis:

Diagnosis is clinical, in the absence of evidence of infections or cyclic neutropenia:

• Regular recurrent fever of early onset.
• Oral aphthous ulcers.
•  Cervical lymphadenopathy.
• Pharyngitis.
•  less commonly, headache, abdominal pain, and arthralgia.

The episodes last 4-6 days, regardless of antipyretic or antibiotic treatment, and often occur with clock-like regularity on 3-6 wk cycles.

Children are in a good health between episodes.

Fever cycles usually stops by the teenage years.

Treatment

• Single dose of Prednisone (0.6-2.0 mg/kg),given orally at the onset of symptoms frequently causes a dramatic resolution of the febrile episode, although it does not prevent future episodes and may actually shorten the interval between episodes.
•  Cimetidine:- given at doses of 20-40 mg/kg/day is effective at preventing recurrences in approximately one-third of cases.
• Tonsillectomy: approximately 50% success reported.
• Colchicine or anakinra may be useful but have an unpredictable response.

Prognosis: is good; most outgrow their symptoms by adolescence.

References:-

• Amanda K. Ombrello and Daniel L. Kastner , Hereditary Periodic Fever Syndromes and Other Systemic Autoinflammatory Diseases,Chapter 163,NELSON TEXTBOOK OF PEDIATRICS, TWENTIETH EDITION 2016
• Clarissa Pilkington, Kiran Nistala, Helen Lachman and Paul Brogan, Rheumatology , GREAT ORMOND STREET HANDBOOK OF PAEDIATRICS, 2nd  ed
• Osama Naga,Rheumatologic Disorders, Periodic Fever ,190-192 Pediatric Board Study Guide, A Last Minute Review
• Sujata Sawhney and Amita Aggarwal, Autoinflammatory Syndromes in Children, Pediatric Rheumatology,546-554 A Clinical Viewpoint,2017
• Ronald M. Laxer ,David D. Sherry  and Philip J. Hashkes,CH10 Autoinflammatory Syndromes,189-208 Pediatric Rheumatology in Clinical Practice,2nd ed 2016