Background Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease occurring in children. Although PFAPA is the most common periodic fever syndrome found in children, there are only a few studies defining the clinical characteristics and the efficacy of treatment strategies among Japanese children. This study aimed to clarify the demographic characteristics and clinical features of patients with PFAPA syndrome and to evaluate treatment efficacy. Methods We retrospectively reviewed the clinical features of children with PFAPA who visited Saitama Children's Medical Center between January and December 2019. We also evaluated treatment strategies and their efficacy; abortive treatment with corticosteroids, prophylaxis with cimetidine or colchicine, and surgical management with tonsillectomy. Results A total of 100 Japanese children (61% male) with PFAPA were included. Median age of onset was 3 years, median duration of fever episodes was 5 days, and median interval between episodes was 4 weeks. The symptoms (frequencies) were pharyngitis (89%), exudate on tonsils (71%), cervical adenitis (50%), and aphthous stomatitis (49%). Approximately 37% of patients took prednisolone for aborting fever attacks, showing a 100% response; 93% were treated with cimetidine, showing an 79.6% response, and 18% were treated with colchicine, showing a 66.7% response. Only one patient underwent tonsillectomy. Conclusions Among Japanese children with PFAPA, 28% of them were ≥5 years with a male predominance. Pharyngitis is the most frequent symptom associated with fever. Cimetidine is suitable for initial therapy because of its safety and efficacy.

Acute encephalopathy/encephalitis is extremely rare and is a severe complication of Kawasaki disease (KD). 1 Acute encephalopathy associated with KD is reportedly resistant to intravenous immunoglobulin (IVIG) therapy, and coronary artery lesions occur more frequently. However, the risk factors for such severe complications remain unclear. Here we report a case of human rhinovirus upper respiratory infection preceded by KD complicated with acute encephalopathy.A 2-year-old boy presented to our hospital with status epilepticus. He had generalized tonic convulsions that were refractory to diazepam and midazolam infusion but finally stopped with thiopental. He was intubated and transferred to our pediatric intensive care unit for further treatment. Three days prior to admission, he had a cough and fever. His past medical history included a breath-holding spell at age 8 months and convulsions with mild gastroenteritis at age 1 year. On admission, his temperature was 37.6 °C and his heart rate was 150 beats per minute (bpm). Notable physical findings were pharyngitis, bilateral cervical lymphadenopathy, and papular rash on his trunk and extremities. Laboratory analysis showed elevated leukocyte count (9,910/µL) and total bilirubin (3.7 mg/dL), aspartate aminotransferase (390 U/L), and C-reactive protein levels (15.56 mg/dL). Serum sodium levels decreased (132 mEq/L). Cerebrospinal fluid (CSF) examination revealed normal results. Blood, urine, and CSF bacterial cultures were negative. On day 4 of his illness, the anticonvulsant was discontinued and he was extubated. On day 5, he developed conjunctival injection, redness of the lips, strawberry tongue, and edematous hands and feet, thus meeting all six major diagnostic criteria for KD. The Kobayashi score for IVIG resistance was 8. He was treated with IVIG (2 g/kg), IV prednisolone (2 mg/kg/day), and oral aspirin (30 mg/kg/day). Figure 1 depicts the patient's clinical course