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Rheumatology International

, Volume 39, Issue 6, pp 1099–1105 | Cite as

Recurrence of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome after tonsillectomy: case-based review

  • Ezgi Deniz BatuEmail author
  • Huseyin Bulut Batu
Case Based Review

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome for which tonsillectomy is a therapeutic option curing the disease in most patients. Recurrence after remission with tonsillectomy is extremely rare. Increasing number of reports on diverse disease manifestations in PFAPA could give us clues about the disease etiopathogenesis. We aimed to describe a patient with recurrence of PFAPA syndrome after tonsillectomy and to review the previous studies including similar cases. We report a 17-year-old boy with PFAPA syndrome who experienced remission for 3 years after tonsillectomy and was later found to harbor an MEFV mutation when the disease relapsed. He responded well to colchicine treatment at relapse. The literature review revealed 14 articles describing 24 similar PFAPA patients. The therapeutic options include single-dose corticosteroids and nonsteroidal anti-inflammatory drugs during attacks, cimetidine, and resurgery. The presented case was the only one heterozygous for an MEFV mutation and treated with colchicine at disease relapse. Albeit rare, the reoccurrence of PFAPA after tonsillectomy could occur. The presence of such patients opposes with the hypothesis that the trigger or immune dysregulation in PFAPA pathogenesis resides in tonsils.

Keywords

PFAPA syndrome Tonsillectomy Relapse Recurrence 

Notes

Author contribution

EDB designed the structure of the article, EDB and HBB performed the literature review, drafted and critically revised the text, and approved the final version of the manuscript.

Funding

No funding was received for this study.

Compliance with ethical standards

No part of the manuscript including the table and figure were published elsewhere.

Conflict of interest

The authors declare no conflict of interest.

Informed consent

Written informed consent was obtained from the presented patient and his parents.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Rheumatology, Department of Pediatrics, Ankara Training and Research HospitalUniversity of Health SciencesAnkaraTurkey
  2. 2.Division of Respiratory Medicine Cardiology, Intensive Care and Sleep Medicine, Department of Internal MedicineAmeos Klinikum am BürgerparkBremerhavenGermany

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