Prediction Of Colchicine Response In Children With Periodic Fever Aphthous Stomatitis Pharyngitis And Cervical Adenitis Syndrome

Abstract

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 favourable 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 was 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.