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揭示埃及血管炎的真相:一项多中心回顾性队列研究,探讨其特征、治疗和结局。

Shedding light on vasculitis in Egypt: a multicenter retrospective cohort study of characteristics, management, and outcome.

机构信息

Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University Hospitals, Saray El Manial Street, El Manial, Cairo, 11956, Egypt.

Internal Medicine and Rheumatology Department, Faculty of Medicine, Tanta University, Tanta, Egypt.

出版信息

Clin Rheumatol. 2019 Jun;38(6):1675-1684. doi: 10.1007/s10067-019-04441-4. Epub 2019 Feb 9.

Abstract

OBJECTIVES

The frequency of different vasculitides and their characteristics vary among different regions. The identification of geographic disparities of disease phenotypes helps the development of international criteria, allowing the classification of patients of different ethnicities. This study aimed to describe the frequency, characteristics, course, response to treatment, and outcome of the different adulthood vasculitides in Egypt.

METHODS

This was a multicenter study in which the medical records of adult Egyptian patients diagnosed with vasculitis between 2002 and 2018 were retrospectively reviewed.

RESULTS

The most frequent vasculitides in Egypt were Behçet's disease (76%), hepatitis C virus vasculitis (13.9%), and granulomatosis with polyangiitis (3.9%). Most patients (73.8%) had a major event at the time of diagnosis. Generalized granulomatosis with polyangiitis was more common than the localized type (90% versus 10%, respectively). The aortic arch and its branches were the most common affected sites of Takayasu arteritis. Of vasculitides, Behçet's disease and giant cell arteritis were associated with the greatest rates of relapse (62.7% and 33.3%, respectively). Delayed diagnosis and permanent organ damage were reported in 69.9% and 68.9% of patients, respectively. A low mortality rate was noted (1.3%).

CONCLUSIONS

The most common types of adulthood vasculitides in Egypt are Behçet's disease, hepatitis C virus vasculitis, and granulomatosis with polyangiitis. Major organ involvement is frequent. Delayed diagnosis and permanent organ damage are common.

摘要

目的

不同血管炎的频率及其特征在不同地区有所不同。识别疾病表型的地域差异有助于制定国际标准,从而对不同种族的患者进行分类。本研究旨在描述埃及成人血管炎的频率、特征、病程、治疗反应和结局。

方法

这是一项多中心研究,回顾性分析了 2002 年至 2018 年间埃及诊断为血管炎的成年患者的病历。

结果

埃及最常见的血管炎为贝赫切特病(76%)、丙型肝炎病毒相关性血管炎(13.9%)和肉芽肿性多血管炎(3.9%)。大多数患者(73.8%)在诊断时存在重大事件。全身性肉芽肿性多血管炎比局限性类型更为常见(分别为 90%和 10%)。主动脉弓及其分支是 Takayasu 动脉炎最常见的受累部位。在血管炎中,贝赫切特病和巨细胞动脉炎的复发率最高(分别为 62.7%和 33.3%)。分别有 69.9%和 68.9%的患者报告存在延迟诊断和永久性器官损害。死亡率较低(1.3%)。

结论

埃及最常见的成人血管炎类型为贝赫切特病、丙型肝炎病毒相关性血管炎和肉芽肿性多血管炎。主要器官受累常见。延迟诊断和永久性器官损害常见。

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