Prediction of Colchicine Response in Children with Periodic Fever Aphthous Stomatitis Pharyngitis and Cervical Adenitis Syndrome

Yıl 2023, Cilt: 10 Sayı: 3, 198 - 203, 19.12.2023

Hakan Kısaoğlu Özge Baba Mukaddes Kalyoncu

https://doi.org/10.47572/muskutd.1327375

Öz

Colchicine prophylaxis is one of the treatment options in management of patients with periodic fever aphthous stomatitis pharyngitis and cervical adenitis (PFAPA) syndrome, but variability exists in response to the treatment. Here we aimed to investigate the efficacy of colchicine prophylaxis and factors associated with a favorable response. Patients diagnosed with PFAPA in whom colchicine was employed before 5 years old age were included. Response to the prophylaxis was assessed by the change of fever intervals and an increase of fever interval >50% after treatment was accepted as favorable response. Complete response was defined as a fever free interval of > 3 months in patients displayed favorable response. Among 41 patients, a favorable response, was observed in 20 (48.8%) patients, and among favorable responders, 8 (19.5%) patients displayed complete response. Of the remaining patients without a favorable response, 9 (22%) demonstrated no response to colchicine prophylaxis. Despite colchicine response was not associated with clinical and laboratory features including MEFV mutations and previous corticosteroid usage, shorter fever intervals were found to be significantly increased the odds of a favorable response to colchicine, which might aid in therapeutic decisions in management of patients with PFAPA.

Anahtar Kelimeler

Fever Intervals, Colchicine, PFAPA, Treatment Response

Periyodik Ateş Aftöz Stomatit Farenjit Servikal Adenit Sendromlu Çocuklarda Kolşisin Yanıtının Belirleyicileri

Öz

Kolşisin profilaksisi, periyodik ateşli aftöz stomatit, farenjit ve servikal adenit (PFAPA) sendromlu hastaların yönetiminde tedavi seçeneklerinden biridir, ancak tedavi yanıtı değişkenlik göstermektedir. Burada kolşisin profilaksisinin etkinliğini ve profilaksiye olumlu yanıtla ilişkili faktörleri araştırmayı amaçladık. Çalışmaya 5 yaşından önce kolşisin tedavisi başlanan PFAPA tanılı hastalar dahil edildi. Profilaktik tedaviye cevap ateşlenme aralıklarındaki değişime göre değerlendirildi ve ateş aralığında> %50 artış olması olumlu yanıt olarak kabul edildi. Olumlu yanıt veren hastalarda ise >3 ay ateşsiz dönem olması tam yanıt olarak kabul edildi. Çalışmaya katılan 41 hasta arasında, 20 (%48.8) hastada olumlu bir yanıt gözlendi ve olumlu yanıt verenler arasında 8 (%19.5) hasta ise tam yanıt verdi. Olumlu yanıt vermeyen diğer hastalardan 9'u (%22) kolşisin profilaksisine yanıtsız olarak değerlendirildi. Kolşisin yanıtının, MEFV mutasyonları ve önceki kortikosteroid kullanımı dahil olmak üzere klinik ve laboratuvar özelliklerle ilişkisi olmamasına rağmen, daha kısa ateş aralıklarının, kolşisine olumlu yanıt verme olasılığını önemli ölçüde artırdığı bulundu. Bu bilgi, PFAPA'lı hastaların tedavisinde terapötik kararlara yardımcı olabilir.

Anahtar Kelimeler

Ateş Aralığı, Kolşisin, PFAPA, Tedavi Yanıtı

Kaynakça

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