白塞病的眼部受累有特点

翻译 武东

摘要 背景：白塞病 （BD）是一种多系统、慢性复发性炎性疾病，是血管炎的一种。眼受累是BD最严重的表现之一，且可见于50%的患者。青年男性患者的眼受累似乎更频繁、更严重，不幸的是，它仍是残疾的一个重要原因。

目的：主要目的是评估BD患者的眼受累发生率，并在近20年进行随访；次要目的是比较不同性别BD患者的眼病发生差异。

方法：对117例BD患者进行了回顾性研究。男/女比例为1.6:1，平均病程为（11 ± 5）年，平均年龄（42 ± 9）岁，最小年龄18岁，最大年龄77，平均发病年龄（25 ± 4）岁，最小发病年龄10岁，最大发病年龄58岁。对所有患者的眼部临床特点及治疗方法进行了回顾性分析。

结果：眼受累的发生率为40％[共47例患者，男29例，女18例；平均发病年龄(29 ± 5)岁]。从首发症状到眼部受累的平均时间间隔为（3 ± 2）年，单眼受累17例（36％），其他均为双眼受累（64％）。眼病发作次数：后葡萄膜炎32次，前葡萄膜炎27次，视网膜血管炎21次，全葡萄膜炎17次。双眼视力丧失1例，一只眼失明3例。31例患者（男28例，女3例）的病程有反复。与女性相比，男性BD患者的后葡萄膜炎（6 / 18 对26/29，P<0 ,0001）和视网膜血管炎（6/18对15/29，p<0.01）的发生率明显增高。

结论：40％的BD患者眼部炎性受累，主要特点为双侧受累。从首发症状到眼受累间平均间隔时间为3年，后葡萄膜炎和视网膜血管炎在男性似乎更多见，且男性的病情复发更多见。

附原文: Background: Beh?et's disease (BD) is a multisystemic, chronic relapsing inflammatory disease classified among the vasculitis. Eye involvement represent one of the most serious manifestation of BD and occurs in half of all patients. It seems more frequent and severe among young males and, unluckily, it still represents a significant cause of morbidity. Objectives: The primary aim of the study was to assess the prevalence of ocular involvement in a cohort of patients with BD, who have been followed in the last twenty years in our Institution. The secondary aim was to compare any possible differences in the prevalence of ocular lesions according to the gender. Methods: One hundred and seventeen patients were retrospectively studied. The males/females, ratio was 1.6:1, with a mean disease duration of 11±5 yrs. Their mean age was 42±9 years (min:18, max:77), while the mean age at disease onset was 25±4 years (min:10, max:58). Prevalence of ocular clinical features and therapeutic approach were retrospectively analysed in all patients. Results: Ocular involvement was observed in 40% (47 patients, 29 males and 18 females; mean age at the onset 29±5 years). The mean time between the first initial symptoms of BD and the onset of eye lesions was 3±2 years; additionally unilateral ocular disease occurred in 17 patients (36%), while the other cases (64%) experienced bilateral involvement. The number of episodes of ocular lesions was the following: 32 posterior uveitis, 27 anterior uveitis, 21 retinal vasculitis, while panuveitis developed in 17 subjects. Loss of vision occurred in both eyes in 1 case and in one eye in 3 patients. In 31 patients the disease course was recurrent (28 males, 3 females). Additionally, comparing the frequency of ocular lesions according to the gender, we observed that males were characterised by a higher prevalence of posterior uveitis (26/29 males versus 6/18 females, p<0 ,0001)and retinal vasculitis (15/29 males versus 6/18 females, p<0.01).conclusion: in our cohort 40% of patients experienced inflammatory eye involvement, that was mainly characterised by bilateral involvement. literature data suggest that ocular involvement generally occurs within few years of disease onset; this is in accordance with our results, that revealed a mean time of 3 years between the first initial symptoms of bd and the onset of eye lesions. posterior panuveitis and retinal vasculitis seem more common in males, who, moreover, are characterised by a more frequent recurrent course.

引自：R. Talarico, A. d'Ascanio, M. Figus, S. Bombardieri. Ocular involvement in Behect’s disease: analysis of a monocentric cohort. Ann Rheum Dis 2010;69(Suppl3):236