CASE REPORT
Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome persisting to adulthood – an example of a diagnostic and therapeutic challenge
1
Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland
2
Department of Immunology, Children’s Memorial Health Institute, Warsaw, Poland
Submission date: 2019-07-08
Final revision date: 2019-08-06
Acceptance date: 2019-09-25
Online publication date: 2019-10-31
Publication date: 2019-11-19
Reumatologia 2019;57(5):292-296
KEYWORDS
polygenic autoinflammatory diseasesadultsperiodic feveraphthous stomatitispharyngitiscervical adenitiscolchicinetreatment
TOPICS
Cytokines, Inflammatory mediators and adaptive immunity in rheumatic diseasesInfection related rheumatoid diseases
ABSTRACT
Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of periodic fever in childhood. Reports of adult patients are sparse. In adults the clinical picture is more heterogeneous than in children, so PFAPA can be a real diagnostic challenge. Data regarding treatment efficacy and disease outcome are available mainly for children, whereas for adult patients they are limited and conflicting. Our aim is to increase the awareness about PFAPA among clinical practitioners. We present a case of PFAPA beginning in childhood and without resolution of symptoms in maturity. In our case the diagnostic delay was 15 years. We treated the patient with a prophylactic dose of colchicine. Colchicine helped to control flares and significantly improved the patient’s quality of life. Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis in adults is a rare disease, but it should be included in the differential diagnosis of fever of unknown origin in adults.
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Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
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