Disease Information for PFAPA/Periodic fever (Marshals benign)

Clinical Manifestations
Signs & Symptoms
Fever Non Infection
Recurrent Abdominal Pain
Palate edema/Soft palate
Palate Erythema/Soft Palate
Cervical adenopathy
Pharyngeal edema/throat swelling
Sore Throat in Children
Sore throat/Pharyngitis
Sore Throat/Throat Pain
Throat Conditions
Cyclic Periodic Recurrent Fevers
Fever and Normal Sed Rate
Fever, intermittant/recurrent
Periodic fever
Quotidian Fever
Disease Progression
Course/Periodic Episodic
Demographics & Risk Factors
Population Group
Population/Pediatrics population
Family History
Family history/Immune defects
Sex & Age Groups
Population/Child-Infant Only
Population/Young child ('Twos')
Associated Diseases & Rule outs
Rule Outs
Aphthous stomatitis Aphthous oral lesions
Pharyngoconjunctival fever/adenovirus
Associated Disease & Complications
Aphthous stomatitis Aphthous oral lesions
Disease Mechanism & Classification
CLASS/Pediatric disorders (ex)
Pathophysiology/Autoinflammatory (non-immune)
Pathophysiology/Multisystem disease
Pathophysiology/Auto-Inflammatory Disorder
PROCESS/Autosomal recessive disorder (ex)
PROCESS/INCIDENCE/Extremely rare disease
PFAPA Periodic fever (Marshals benign), Synonym/Marshals syndrome/FAPA PF syndrome
Drug Therapy - Indication
RX/Cimetidine (Tagamet)
RX/Prednisone (Deltasone)
RX/Rilonacept IL-1 Blocker (Arcalyst)
Surgical Procedures or Treatments

Marshall"s Syndrome of Periodic fever-Aphthous stomatitis-pharyngitius-adenitis; PFAPA syndrome; the age of onset was less than 5 years in most cases and the patients presented fever, pharyngitis,, cervical adenitis, stomatitis, malaise, headache, abdominal pain, and nausea/vomiting , Afebrile intervals were 3-2 +/- 2-4 months and increased with age; The time from initial onset to final episode was 3 years 7 months; Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine;The general outcome was good ;. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years; It was concluded that typical PFAPA syndrome is benign; and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes ; that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA;

The classic periodic fever syndrome is cyclic neutropenia (neutropenia followed by infections and fever that recur every 21 days);A new periodic fever syndrome PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) has been characterized over the past decade; PFAPA is defined clinically, because specific laboratory abnormalities have not been found; The clinical characteristic of PFAPA is high fevers (usually 40-0 degrees C to 40-6 degrees C) recurring at fixed intervals every 2 to 8 weeks; The fevers last for about 4 days, then resolve spontaneously; Associated with the fevers are aphthous stomatitis in 70% of patients, pharyngitis in 72% of patients, and cervical adenitis in 88% of patients ;PFAPA is not familial and begins before the age of 5 years


External Links Related to PFAPA/Periodic fever (Marshals benign)
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)