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Genetic and Rare Diseases Information Center (GARD)

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Periodic fever, aphthous stomatitis, pharyngitis and adenitis


Other Names for this Disease
  • Marshall Syndrome
  • PFAPA
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Your Question

What is PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome? What causes it? Are there any support groups? Are there any research studies?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA)?

Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) is a periodic disease, which is a heterogeneous group of disorders characterized by short episodes of illness that regularly recur for several years alternated with healthy periods.[1] PFAPA is characterized by high fevers lasting three to six days and recurring every 21 to 28 days, accompanied by some or all of the signs noted in its name, namely mouth sores (aphthous stomatitis), sore throat (pharyngitis), and enlarged lymph nodes (cervical adenitis). The syndrome usually occurs in children younger than five years; although it has been reported in children up to 13 years.[2] The syndrome is sporadic and non-hereditary.[3] The course of PFAPA can be persistent for years before spontaneous, full resolution.[4]
Last updated: 10/7/2013

What causes periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA)?

The cause of PFAPA is unknown, although viral or autoimmune causes have been suggested.[1]
Last updated: 8/16/2011

How can I learn about research involving periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA)?

The National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. While no studies involving PFAPA specifically are listed at this time, there is a trial titled Familial Mediterranean Fever and Related Disorders: Genetics and Disease Characteristics. To learn more click on the links above. Review the trials "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. You can check ClinicalTrials.gov often for regular updates.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH) by calling 1-800-411-1222 to speak with a specialist, who can help you determine if you are eligible for any clinical trials. 

ClinicalTrials.gov provides a helpful guide for information about participating in a clinical trial. Resources for travel and lodging assistance are listed on the National Center for Advancing Translational Sciences Office of Rare Diseases Research (NCATS-ORDR) website, part of the National Institutes of Health (NIH).

Last updated: 10/7/2013

Are there any advocacy organizations for individuals and families with periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA)?

Additional information and supportive resources for PFAPA can be obtained from the following organization.

NOMID Alliance, Inc.
P.O. Box 590354
San Francisco CA 94118
Telephone: 415-831-8782
E-mail: karen.nomidalliance.org@gmail.com
Web site: http://www.nomidalliance.org/


 

Last updated: 12/7/2012

References
  • Berlucchi M, Nicolai P. Marshall’s syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. Orphanet encyclopedia. 2004; http://www.orpha.net/data/patho/GB/uk-PFAPA.pdf. Accessed 10/7/2013.
  • Ovetchkine P, Bry ML, Reinert P. [Marshall syndrome: results of a retrospective national survey].. Arch Pediatr. 2000; http://www.ncbi.nlm.nih.gov/pubmed/10941483. Accessed 10/7/2013.
  • Kurtaran H, Karadað A, Catal F, Aktaþ D. PFAPA syndrome: a rare cause of periodic fever. Turk J Pediatr. 2004; http://www.ncbi.nlm.nih.gov/pubmed/15641271. Accessed 10/7/2013.
  • Long S.. Tonsillectomy as treatment of PFAPA syndrome. Journal of Pediatrics. 2007;