Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome: Current Literature Review with a Case Report

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis syndrome (PFAPA) is a frequently seen, important medical condition, which must be kept in mind in periodic fever etiology. Although its etiology is not clearly understood, autoimmune etiology is suspected due to response to steroids. There is no specific test for the diagnosis of disease. Diagnosis is based on symptoms and physical examination. Although medical treatment is the first choice for the management of the disease, tonsillectomy take over in treatment options for termination of attacks and permanent results. We have presented a patient that was performed tonsillectomy in our clinic for frequent attacks despite recurrent medical therapy with diagnosis of PFAPA with review of current literature.

Periyodik Ateş, Aftöz Stomatit, Farenjit ve Servikal Adenit Sendromu: Bir Olgu Nedeniyle Güncel Literatürün Gözden Geçirilmesi

Periyodik Ateş, Aftöz Stomatit, Farenjit ve Servikal Adenit sendromu (PFAPA), sık izlenen ve periyodik ateş etiyolojisinde akılda bulundurulması gereken önemli bir sendromdur. Kesin olarak etiyopatogenezi bilinmese de steroid grubu ilaçlara cevap vermesi nedeni ile otoimmün bir hastalık olarak değerlendirilmektedir. Tanıda hastalığa özgü test mevcut değildir. Tanı, semptomlara ve fizik muayeneye dayanmaktadır. Medikal tedavi ilk seçenek olarak gözükse de atakların kesilmesi ve kalıcı yanıtların elde edilmesi nedeni ile tonsillektomi belirgin şekilde ön plana çıkmaktadır. Kliniğimizde PFAPA tanısı ile tekrarlayan medikal tedavi almasına rağmen atakların sık olarak tekrarlaması nedeni ile tonsillektomi uygulanan bir hasta eşliğinde güncel literatür gözden geçirilmiştir. Periodic

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Thomas KT, Edwards KM. Periodic fever syndrome. Pediatr Infect Dis J 1999; 18: 68-9.

Kuppersmith RB, Schams ST, Rosenfeld RM. Extracts from The Cochrane Library: Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Otolaryngol Head Neck Surg 2010; 143: 473-5.

Hofer M, Pillet P, Cochard MM, Berg S, Krol P, Kone-Paut I, et al. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatology (Oxford) 2014; 53: 1125-9.

Akelma AZ, Cizmeci MN, Kanburoglu MK, Mete E, Bozkaya D, Tufan N, et al. Is PFAPA syndrome really a sporadic disorder or is it genetic? Med Hypotheses 2013; 81: 279-81.

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Meandros Medical And Dental Journal-Cover
  • ISSN: 2149-9063
  • Başlangıç: 2000
  • Yayıncı: Erkan Mor
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