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Performance of recently proposed periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria in a region endemic for familial Mediterranean fever

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Abstract

The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an auto-inflammatory condition characterized by recurrent episodes of fever accompanied by aphthosis, cervical adenitis, and pharyngitis. Diagnosis of PFAPA could be challenging due to clinic overlap with familial Mediterranean fever (FMF). An international consensus has been established recently, to define a new set of classification criteria for PFAPA syndrome. We aimed to evaluate the performance of recently proposed PFAPA criteria, to assess their utility in FMF regions. Patients diagnosed with PFAPA syndrome, FMF, and juvenile idiopathic arthritis (JIA) were included. Two investigators blindly evaluated all of patients for the newly proposed PFAPA criteria. A total of 542 patients (322 with PFAPA syndrome, 118 FMF and 102 JIA) were evaluated. Mean age of patients was 6.6 ± 2.81, 12.75 ± 3.9, and 12.42 ± 4.8 years for PFAPA, FMF, and JIA, respectively. We found quite high sensitivity (89.7%) but insufficient specificity of newly proposed PFAPA criteria (69.5%). When applied to control patients separately, specificity was found to be 61% and 79.4% for FMF and JIA patients, respectively. Positive predictive value was 81%, while negative predictive value was 82%. Recently proposed PFAPA criteria have satisfactory sensitivity, but its specificity is still under expectation. There is a need for a distinctive criterion between PFAPA syndrome and FMF, in FMF endemic regions, e.g., cryptic tonsillitis rapidly responsive to single dose of glucocorticoids. Further studies with higher patients’ number in different regions are needed.

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey

    Amra Adrovic, Mehmet Yıldız, Melisa Kanber, Ipek Ulkersoy, Neslihan Gucuyener, Oya Koker, Sezgin Sahin, Kenan Barut & Ozgur Kasapcopur

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  1. Amra Adrovic
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  2. Mehmet Yıldız
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  3. Melisa Kanber
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  4. Ipek Ulkersoy
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  5. Neslihan Gucuyener
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  6. Oya Koker
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  7. Sezgin Sahin
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  8. Kenan Barut
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  9. Ozgur Kasapcopur
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Contributions

AA contributed to the conception and design of the study, data analysis, and manuscript writing. MY had substantial contribution to conception and design of the study and to data analysis. MK, IU, and NG contributed to data collection and data input. OK contributed to data analysis and manuscript writing. SS and KB contributed to data analysis and critical revisions. OK made final contributions to the conception and design, revision of draft manuscript, and approval of the manuscript to be submitted for publication. All authors read and approved the final version of the manuscript.

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Correspondence to Ozgur Kasapcopur.

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Adrovic, A., Yıldız, M., Kanber, M. et al. Performance of recently proposed periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria in a region endemic for familial Mediterranean fever. Rheumatol Int 40, 91–96 (2020). https://doi.org/10.1007/s00296-019-04362-0

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