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5/2019 vol. 57
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OPIS PRZYPADKU
Zespół PFAPA rozpoznany w wieku dorosłym- wyzwanie diagnostyczne i terapeutyczne- opis przypadku i przegląd literatury
Ewa Więsik-Szewczyk 1
,
 
Beata Wolska-Kuśnierz 2
,
 
Karina Jahnz-Różyk 1
 
 
 
Więcej
Ukryj
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
 
 
Data nadesłania: 08-07-2019
 
 
Data ostatniej rewizji: 06-08-2019
 
 
Data akceptacji: 25-09-2019
 
 
Data publikacji online: 31-10-2019
 
 
Data publikacji: 19-11-2019
 
 
Reumatologia 2019;57(5):292-296
DOI: https://doi.org/10.5114/reum.2019.89523
 
 
Artykuł (PDF)
Referencje (33)
 
SŁOWA KLUCZOWE
polygenic autoinflammatory diseases
adults
periodic fever
aphthous stomatitis
pharyngitis
cervical adenitis
colchicine
treatment
 
DZIEDZINY
Cytokiny, mediatory zapalenia i odporność w chorobach reumatycznych
Powiązane z infekcją choroby reumatyczne
 
STRESZCZENIE
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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