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白塞病的临床表现及多系统受累情况:发病性别与年龄的影响

Mode of presentation and multisystem involvement in Behçet's disease: the influence of sex and age of disease onset.

作者信息

Krause I, Uziel Y, Guedj D, Mukamel M, Molad Y, Amit M, Weinberger A

机构信息

Department of Internal Medicine B, Rabin Medical Center, Beilinson Campus, Petah-Tiqva, Israel.

出版信息

J Rheumatol. 1998 Aug;25(8):1566-9.

PMID:9712102
Abstract

OBJECTIVE

To evaluate the correlation between the age at onset of Behcet's disease (BD) and sex distribution, mode of disease appearance, and number of organs involved during the disease.

METHODS

BD was defined according to the International Study Group criteria. Data from medical files and from patient interviews were collected. Results were analyzed for children and adults, according to age at disease onset. A systemic involvement index was calculated as the sum of visceral organ systems involved.

RESULTS

Fifty-nine patients with BD were studied, 26 male and 33 female. The mean age at disease onset was 8.4 +/- 4.5 years in children and 29.8 +/- 7.9 years in adults. The age of onset was significantly lower in male versus female patients. BD presented in children almost entirely as recurrent aphthous stomatitis, while in adults, less than one-third of patients presented first with oral ulcers. The mean age at disease onset of patients who presented first with oral ulcers was significantly lower than the age of patients presenting first with non-oral aphthosis. The mean systemic involvement index was higher in adult onset than in juvenile onset disease. A significant linear correlation was found between age of disease onset and total number of visceral organ systems involved.

CONCLUSION

BD was observed to occur earlier in males than in females. The first manifestation of BD in children is almost exclusively in the form of oral ulcers, while older patients have a large proportion of non-oral aphthosis as their first disease manifestation. Disease onset at an older age is positively correlated with increased disease spectrum.

摘要

目的

评估白塞病(BD)的发病年龄与性别分布、疾病表现方式以及疾病期间受累器官数量之间的相关性。

方法

根据国际研究组标准定义BD。收集病历和患者访谈的数据。根据发病年龄对儿童和成人的结果进行分析。计算全身受累指数,即受累内脏器官系统的总和。

结果

研究了59例BD患者,其中男性26例,女性33例。儿童发病的平均年龄为8.4±4.5岁,成人为29.8±7.9岁。男性患者的发病年龄明显低于女性患者。BD在儿童中几乎完全表现为复发性口腔溃疡,而在成人中,不到三分之一的患者首先出现口腔溃疡。首先出现口腔溃疡的患者的发病平均年龄明显低于首先出现非口腔口疮的患者。成人发病的平均全身受累指数高于青少年发病。发现发病年龄与受累内脏器官系统总数之间存在显著的线性相关性。

结论

观察到BD在男性中比在女性中发病更早。BD在儿童中的首发表现几乎完全是口腔溃疡的形式,而老年患者有很大比例的非口腔口疮作为其首发疾病表现。发病年龄较大与疾病谱增加呈正相关。

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