Güngörer Vildan, Ünal Dilara, Çakan Mustafa, Ayduran Semra, Gül Ümit, Zora Hatice Kübra, Öner Nimet, Köker Oya, Uçak Kübra, Şahin Nihal, Demir Selcan, Demirbaş Bahar, Özdel Semanur, Yüksel Selçuk, Kilic Sara Sebnem, Bilginer Yelda, Kasapçopur Özgür, Özen Seza, Çelikel Acar Banu
Department of Pediatric Rheumatology, University of Health Sciences, Ankara City Hospital, Ankara, Türkiye.
Department of Pediatric Rheumatology, University of Health Sciences, Gülhane Faculty of Medicine, Ankara, Türkiye.
Clin Rheumatol. 2025 Sep 18. doi: 10.1007/s10067-025-07682-8.
INTRODUCTION/OBJECTIVES: Syndrome of undifferentiated recurrent fever (SURF) is an autoinflammatory disorder that is recognised in an increasing number of patients. In this study, we aimed to assess the data of SURF patients from the main reference centres in our country.
Data for this retrospective multicentre observational cohort study were obtained from the records of SURF patients aged 0-18 years who were followed up in 10 pediatric rheumatology clinics in Türkiye between 2010 and June 2023. Patients with recurrent fever that could not be explained by periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) and hereditary recurrent fevers and had no other cause were included in the study.
Of the 134 patients included in the study, 74 (55.2%) were male. The median age at diagnosis was 67 months. The most common symptom was abdominal pain in 98 (73.1%), arthralgia in 82 (61.2%), malaise in 77 (57.5%). The age at symptom onset was ≤ 5 years in 109 patients (81.3%). Pharyngitis was more common symptom in children aged ≤ 5 years (p = 0.008), headache, arthralgia, chest pain were more common findings in children > 5 years (p = 0.008, p = 0.032, p = 0.045). There were 113 patients receiving colchicine alone or in combination therapy and 74.3% of them achieved complete or partial remission. The presence of abdominal pain (p = 0.021, OR = 0.254) increased the remission rate with colchicine.
SURF patients present with a wide range of clinical manifestations. Distinguishing between SURF and PFAPA is not concrete. Further omics studies will enlighten whether there is a true group of SURF. Key Points • SURF is an autoinflammatory disease that is becoming increasingly recognised. • The clinical manifestations of SURF are quite heterogeneous. • Colchicine and anti-IL-1 treatment is effective in most SURF patients. • It is controversial whether it should be called SURF or PFAPA-like syndrome, especially in children aged ≤ 5 years.
引言/目的：未分化型复发性发热综合征（SURF）是一种自身炎症性疾病，越来越多的患者被诊断出患有该疾病。在本研究中，我们旨在评估我国主要参考中心的SURF患者数据。
这项回顾性多中心观察性队列研究的数据来自2010年至2023年6月在土耳其10家儿科风湿病诊所接受随访的0至18岁SURF患者的记录。研究纳入了无法用周期性发热、口疮性口炎、咽炎和颈淋巴结炎（PFAPA）以及遗传性复发性发热解释且无其他病因的复发性发热患者。
在纳入研究的134例患者中，74例（55.2%）为男性。诊断时的中位年龄为67个月。最常见的症状是腹痛，共98例（73.1%）；关节痛，82例（61.2%）；不适，77例（57.5%）。109例患者（81.3%）症状出现时年龄≤5岁。咽炎在≤5岁儿童中更常见（p = 0.008），头痛、关节痛、胸痛在＞5岁儿童中更常见（p = 0.008、p = 0.032、p = 0.045）。113例患者接受了单独或联合秋水仙碱治疗，其中74.3%实现了完全或部分缓解。腹痛的存在（p = 0.021，OR = 0.254）提高了秋水仙碱治疗的缓解率。
SURF患者表现出广泛的临床表现。区分SURF和PFAPA并不明确。进一步的组学研究将揭示是否存在真正的SURF群体。要点 • SURF是一种越来越被认可的自身炎症性疾病。 • SURF的临床表现非常异质性。 • 秋水仙碱和抗IL-1治疗对大多数SURF患者有效。 • 是否应称为SURF或PFAPA样综合征存在争议，尤其是在≤5岁的儿童中。
