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秋水仙碱对贝赫切特谱系障碍引起的口腔溃疡的疗效,这些障碍包括特发性复发性阿弗他口炎、周期性发热、口疮性口炎、咽炎和颈淋巴结炎综合征(PFAPA)以及贝赫切特病。

Colchicine efficacy on oral ulcers caused by Behçet's spectrum disorders including idiopathic recurrent aphthous stomatitis, PFAPA, and Behçet's Disease.

作者信息

Aydin Zelal, Kaya Feray, Kucuk Elif, Koru Lutfiye, Dizman Eda Nur, Dursun Hatice Kubra, Balci Merve Ozen, Haslak Fatih, Ozturk Kubra

机构信息

Department of Pediatric Rheumatology, Istanbul Medeniyet University, Istanbul, Turkey.

出版信息

Eur J Pediatr. 2025 Jul 31;184(8):516. doi: 10.1007/s00431-025-06363-7.

Abstract

UNLABELLED

Recurrent aphthous stomatitis (RAS) is a common condition in pediatric age. Since there are significant genetic commonalities among idiopathic cases, periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA), and Behçet's disease (BD), they were proposed to be considered in one clinical spectrum termed Behçet's Spectrum Disorders (BSDs). This retrospective study aimed to evaluate the efficacy of colchicine on oral ulcers in children with BSD related RAS (BSD-RAS). Secondary aim was to compare the disease groups in terms of clinical and laboratory benefits. Data at admission and one year-after the treatment initiation were compared. A total of 317 patients with RAS were evaluated, and 186 patients classified as BSD-RAS (Female: n = 105, 56%). The median age at symptom onset was 5 (0.5-16.5) years. Colchicine was started to 142 (76%) patients. We observed a significant reduction in the frequency and duration of oral ulcers, as well as in the visual analogue scale (VAS) scores reported by patients/families and physicians, and the erythrocyte sedimentation rate during symptom-free periods across all three groups. In patients with PFAPA, both difference between initial and final recorded values (Δ) patient/families and Δ physician VAS scores were significantly higher than in other groups (p < 0.001). Furthermore, both Δ duration (p = 0.005) and Δ frequency of oral aphthae (p = 0.012) were significantly higher in idiopathic cases than those with PFAPA.

CONCLUSION

These findings demonstrate a substantial reduction in both clinical symptoms and laboratory markers following colchicine treatment, supporting its effectiveness as a therapeutic option for BSD-RAS.

WHAT IS KNOWN

•Recurrent aphthous stomatitis, PFAPA, and Behçet's disease may manifest with similar oral ulcers in pediatric patients. •Colchicine is frequently used in this clinical spectrum known as Behçet's Spectrum Disorders, although there is a scarcity of pediatric data.

WHAT IS NEW

•Colchicine treatment significantly improved clinical and laboratory outcomes across all groups. •Idiopathic group had greater reductions in oral ulcer frequency and duration while PFAPA associated cases showed highest improvement in visual analogue scale scores.

摘要

未标注

复发性阿弗他口炎(RAS)是儿童期的常见病症。由于特发性病例、周期性发热、阿弗他口炎、咽炎和腺炎综合征(PFAPA)以及白塞病(BD)之间存在显著的遗传共性,它们被提议被视为一个临床谱系,称为白塞谱系障碍(BSD)。这项回顾性研究旨在评估秋水仙碱对患有BSD相关RAS(BSD-RAS)的儿童口腔溃疡的疗效。次要目的是在临床和实验室获益方面比较疾病组。比较了入院时和治疗开始后一年的数据。共评估了317例RAS患者,其中186例被归类为BSD-RAS(女性:n = 105,56%)。症状出现时的中位年龄为5(0.5 - 16.5)岁。142例(76%)患者开始使用秋水仙碱。我们观察到所有三组患者口腔溃疡的频率和持续时间、患者/家属及医生报告的视觉模拟量表(VAS)评分以及无症状期的红细胞沉降率均显著降低。在PFAPA患者中,患者/家属和医生VAS评分的初始值与最终记录值之差(Δ)均显著高于其他组(p < 0.001)。此外,特发性病例口腔溃疡的Δ持续时间(p = 0.005)和Δ频率(p = 0.012)均显著高于PFAPA患者。

结论

这些发现表明秋水仙碱治疗后临床症状和实验室指标均显著降低,支持其作为BSD-RAS治疗选择的有效性。

已知信息

•复发性阿弗他口炎、PFAPA和白塞病在儿科患者中可能表现出相似的口腔溃疡。•秋水仙碱常用于这个被称为白塞谱系障碍的临床谱系,尽管儿科数据匮乏。

新发现

•秋水仙碱治疗显著改善了所有组的临床和实验室结果。•特发性组口腔溃疡的频率和持续时间降低幅度更大,而PFAPA相关病例的视觉模拟量表评分改善最为明显。

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