Rydenman Karin, Berg Stefan, Karlsson-Bengtsson Anna, Fasth Anders, Wekell Per
NU-sjukvården - Barn- och ungdomskliniken Uddevalla, Sweden NU-sjukvården - Barn- och ungdomskliniken Uddevalla, Sweden.
Institutionen för kliniska vetenskaper, Sahlgrenska Akademin, Göteborgs Universitet - Avdelningen för pediatrik Göteborg, Sweden Institutionen för kliniska vetenskaper, Sahlgrenska Akademin, Göteborgs Universitet - Avdelningen för pediatrik Göteborg, Sweden.
Lakartidningen. 2019 Oct 29;116:FP9U.
Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is the most common autoinflammatory disorder among children in many parts of the world and an important differential diagnosis in children presenting with recurrent fever episodes. Commonly, PFAPA has an onset under the age of 5 years. Fever episodes in PFAPA usually last 3-6 days and are associated with one or more of the cardinal symptoms aphthous stomatitis, pharyngitis and cervical adenitis. The fever episodes typically recur with an interval of 3-6 weeks, often with a striking regularity. During the episodes, the patient has elevated inflammatory variables such as CRP and serum amyloid A (SAA) and may sometimes have additional symptoms such as abdominal pain, nausea and leg pain. Between the fever episodes, the patient is typically free of symptoms with normalized inflammatory variables and grows normally. Awareness and recognition of PFAPA is key to providing the patient with adequate treatment and avoiding misdiagnosis.
周期性发热、口疮性口炎、咽炎和颈淋巴结炎（PFAPA）综合征是世界许多地区儿童中最常见的自身炎症性疾病，也是反复发热发作儿童的重要鉴别诊断疾病。通常，PFAPA在5岁以下发病。PFAPA的发热发作通常持续3 - 6天，并伴有口疮性口炎、咽炎和颈淋巴结炎中的一种或多种主要症状。发热发作通常以3 - 6周的间隔复发，常常具有显著的规律性。在发作期间，患者的炎症指标如CRP和血清淀粉样蛋白A（SAA）会升高，有时还可能有腹痛、恶心和腿痛等其他症状。在发热发作间期，患者通常没有症状，炎症指标恢复正常，生长发育正常。认识和识别PFAPA是为患者提供适当治疗并避免误诊的关键。
