Rheumatology International

, Volume 39, Issue 1, pp 29–36 | Cite as

Familial Mediterranean fever and periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: shared features and main differences

  • Amra Adrovic
  • Sezgin Sahin
  • Kenan Barut
  • Ozgur Kasapcopur


Autoinflammatory diseases are characterized by fever attacks of varying durations, associated with variety of symptoms including abdominal pain, lymphadenopathy, polyserositis, arthritis, etc. Despite the diversity of the clinical presentation, there are some common features that make the differential diagnosis of the autoinflammatory diseases challenging. Familial Mediterranean fever (FMF) is the most commonly seen autoinflammatory conditions, followed by syndrome associated with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA). In this review, we aim to evaluate disease characteristics that make a diagnosis of FMF and PFAPA challenging, especially in a regions endemic for FMF. The ethnicity of patient, the regularity of the disease attacks, and the involvement of the upper respiratory systems and symphonies could be helpful in differential diagnosis. Current data from the literature suggest the use of biological agents as an alternative for patients with FMF and PFAPA who are non-responder classic treatment options. More controlled studies are needed to evaluate the efficacy and safety of this strategy.


Familial Mediterranean fever PFAPA syndrome Colchicine Recurrent fever Anti-interleukin-1 agents 


Author contributions

OK made a design of the paper. AA, SS, and KB provided the search of the literature and wrote the paper. OK and KB made a critical evaluation of the data collection and interpretation. SS designed a figure. All of the authors additionally revised the final version of the manuscript.


This review was not funded by any person or corporation.

Compliance with ethical standards

Conflict of interest

Author Amra Adrovic declares that she has no conflict of interest. Author Sezgin Sahin declares that he has no conflict of interest. Author Kenan Barut declares that she has no conflict of interest. Author Ozgur Kasapcopur declares that he has receiving advisory board and speaker fees from Novartis.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatric Rheumatology, Cerrahpasa Medical SchoolIstanbul UniversityIstanbulTurkey

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