Department of Pediatrics, University of Health Scienes Dr. Behcet Uz Children's Education and Research Hospital, İsmet Kaptan Neighborhood, Sezer Doğan Street, 11, 35210, Konak/Izmir, Turkey.
Department of Pediatric Immunology and Allergy, University of Health Scienes Dr. Behcet Uz Children's Education and Research Hospital, Izmir, Turkey.
Clin Rheumatol. 2021 Aug;40(8):3219-3225. doi: 10.1007/s10067-021-05652-4. Epub 2021 Feb 22.
The aim of this study is to evaluate our patients with the newly developed international severity score for FMF (ISSF) and make comparisons with the literature.
This cross-sectional study included patients with FMF, were between 6 months and 18 years old, and were using colchicine/colchicine+IL-1 inhibitor for at least 6 months. The patients were classified as mild, intermediate, and severe based on their scores between 1 and 10. In addition to scoring, those who have additional pathological or silent mutation were compared based on these classifications.
Our patients consist of 88 women 72 men total 160. The mean age, age of onset, and age of diagnosis were 12 ± 4.3, 4.9 ± 3.7, and 7.5 ± 4 years, respectively, and the mean diagnostic delay was 2.6 ± 3years. When our large patient population is evaluated with ISSF, the disease severity is mostly intermediate. According to ISSF, mild, intermediate, and severe diseases were 21.3%, 70.5%, and 8.1%, respectively. The duration of attacks shorten with increasing age (p > 0.05), and there is an increase in the involvement of other organs during the attacks among patients older than 7 years.
ISSF appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients. Key Points • Our article is the first study to evaluate ISSF performance in the pediatric population. • International severity score for FMF (ISSF) appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients. • We think that the addition of pathogenic mutations and inheritance model to the ISSF scoring system as important determinants of disease severity, especially when comparing different patients, can be evaluated in future studies.
本研究旨在评估新开发的纤维肌痛国际严重程度评分（ISSF）在我们患者中的表现，并与文献进行比较。
这是一项横断面研究，纳入了使用秋水仙碱/秋水仙碱+IL-1 抑制剂至少 6 个月的、年龄在 6 个月至 18 岁之间的纤维肌痛患者。根据评分（1 至 10 分）将患者分为轻度、中度和重度。除评分外，还根据这些分类比较了有额外病理或沉默突变的患者。
我们的患者共 160 例，其中女性 88 例，男性 72 例。平均年龄、发病年龄和诊断年龄分别为 12 ± 4.3 岁、4.9 ± 3.7 岁和 7.5 ± 4 岁，平均诊断延迟时间为 2.6 ± 3 年。当我们用 ISSF 评估大型患者群体时，疾病严重程度主要为中度。根据 ISSF，轻度、中度和重度疾病的比例分别为 21.3%、70.5%和 8.1%。攻击的持续时间随着年龄的增长而缩短（p > 0.05），7 岁以上患者在攻击期间其他器官受累的情况增加。
ISSF 似乎是一种适合儿科纤维肌痛患者疾病严重程度随访的有效工具。关键点：• 我们的文章是第一项评估 ISSF 在儿科人群中表现的研究。• 纤维肌痛国际严重程度评分（ISSF）似乎是一种适合儿科纤维肌痛患者疾病严重程度随访的有效工具。• 我们认为，在 ISSF 评分系统中添加致病性突变和遗传模型作为疾病严重程度的重要决定因素，特别是在比较不同患者时，可以在未来的研究中进行评估。
