Herrero-Morant Alba, Martín-Varillas José Luis, Álvarez-Reguera Carmen, Sánchez-Bilbao Lara, Martínez-López David, Suárez-Amorín Guillermo, Fernández-Ramón Raúl, Ferraz-Amaro Iván, Castañeda Santos, Hernández José L, Blanco Ricardo
Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Immunopathology Group-IDIVAL, Avda. Valdecilla s/n., 39008 Santander, Cantabria, Spain.
Rheumatology, Hospital de Laredo, 39770 Laredo, Cantabria, Spain.
J Clin Med. 2024 Sep 5;13(17):5270. doi: 10.3390/jcm13175270.
: Neuro-Behçet's disease (NBD) is one of the most severe complications of Behçet's disease (BD). The incidence of NBD varies widely worldwide. This study aimed to estimate its current incidence in Northern Spain. : This was a retrospective population-based cohort study of 120 patients in Northern Spain diagnosed with BD according to the 2013 International Criteria for BD (ICBD) between 1 January 1999 and 31 December 2019. NBD diagnoses were made according to International Consensus Recommendation (ICR) criteria. Overall, 96 patients were included, and their demographic and clinical data were collected. The incidence of NBD was estimated by age, gender, and year of diagnosis between 1999-2019. : NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44 ± 13.9 years). HLA-B51 was positive in 5 of 13 (38.4%) cases tested. A total of 10 (43.5%) patients had parenchymatous NBD, 10 (43.5%) had non-parenchymatous NBD, and 3 (13%) had mixed NBD. Incidence during the study period was 0.13 (95% CI, 0.11-0.26) per 100,000 people-years. There were no significant differences in gender in the incidence rate stratified by age ( > 0.05). Furthermore, there was a linear relationship with a mild decrease in age at diagnosis over time. : Epidemiological characteristics of NBD in Northern Spain are similar to those of neighboring countries, except female gender predominance.
神经白塞病（NBD）是白塞病（BD）最严重的并发症之一。NBD在全球的发病率差异很大。本研究旨在估计其目前在西班牙北部的发病率。：这是一项基于人群的回顾性队列研究，研究对象为1999年1月1日至2019年12月31日期间在西班牙北部根据2013年白塞病国际标准（ICBD）诊断为BD的120例患者。NBD的诊断依据国际共识推荐（ICR）标准。总体而言，纳入了96例患者，并收集了他们的人口统计学和临床数据。通过年龄、性别以及1999 - 2019年的诊断年份来估计NBD的发病率。：96例患者中有23例（24%）被诊断为NBD（15名女性/8名男性）（平均年龄：44±13.9岁）。13例检测病例中有5例（38.4%）HLA - B51呈阳性。共有10例（43.5%）患者患有实质性NBD，10例（43.5%）患有非实质性NBD，3例（13%）患有混合型NBD。研究期间的发病率为每10万人年0.13（95%置信区间，0.11 - 0.26）。按年龄分层的发病率在性别上无显著差异（>0.05）。此外，随着时间推移，诊断年龄呈轻度下降的线性关系。：西班牙北部NBD的流行病学特征与邻国相似，但女性占主导地位除外。
