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系统性硬化症中的胃窦血管扩张症:人口统计学和疾病预测因素。

Gastric antral vascular ectasia in systemic sclerosis: demographics and disease predictors.

机构信息

Division of Rheumatology, Georgetown University, Washington, DC, USA.

出版信息

J Rheumatol. 2010 Mar;37(3):603-7. doi: 10.3899/jrheum.090600. Epub 2010 Jan 15.

Abstract

OBJECTIVES

To evaluate patients with systemic sclerosis (SSc) who have gastric antral vascular ectasia (GAVE), to further characterize this disease association, and to identify factors that may predict which patients with SSc are at greatest risk for the development of GAVE.

METHODS

Patients with a diagnosis of both SSc and GAVE were identified from the Division of Rheumatology at Georgetown University and Thomas Jefferson University. A chart review was conducted to obtain the demographic data.

RESULTS

Twenty-eight patients were included in this analysis, including 17 with diffuse cutaneous (dcSSc) and 11 with limited cutaneous SSc (lcSSc). The mean disease duration at diagnosis with GAVE was 21.5 months for dcSSc and 84.3 months for lcSSc (p = 0.025). Seventy-six percent of patients with dcSSc developed GAVE within 18 months of first scleroderma symptom onset. Over half of patients with early GAVE also had rapidly progressive cutaneous disease. Only 4% had antitopoisomerase I antibody. Although only 1 patient was tested and had positive RNA polymerase (RNAP) III, RNAP III may be overrepresented in this GAVE population. Mean hematocrit levels were 23.8% in dcSSc and 29% in lcSSc.

CONCLUSION

dcSSc is associated with earlier development of GAVE, as well as more severe anemia requiring more therapeutic interventions. Rapid progression of cutaneous disease may suggest earlier development of GAVE. Absence of antitopoisomerase I antibodies and presence of antibodies to RNAP III/speckled antinuclear antibody pattern may be useful to identify the subset of patients with SSc with increased risk for GAVE.

摘要

目的

评估患有系统性硬化症(SSc)合并胃底贲门血管扩张症(GAVE)的患者,进一步阐明这种疾病的相关性,并确定可能预测哪些 SSc 患者发生 GAVE 的风险最大的因素。

方法

从乔治敦大学和托马斯杰斐逊大学的风湿病科确定了同时患有 SSc 和 GAVE 的患者。进行了病历回顾以获取人口统计学数据。

结果

本分析共纳入 28 例患者,其中 17 例为弥漫性皮肤型(dcSSc),11 例为局限性皮肤型 SSc(lcSSc)。dcSSc 患者诊断为 GAVE 的平均病程为 21.5 个月,lcSSc 为 84.3 个月(p=0.025)。76%的 dcSSc 患者在出现硬皮病症状后 18 个月内发生 GAVE。半数以上早期 GAVE 患者也存在快速进展性皮肤疾病。仅 4%的患者存在抗拓扑异构酶 I 抗体。尽管只有 1 例患者接受了 RNA 聚合酶(RNAP)III 检测且为阳性,但在该 GAVE 人群中,RNAP III 可能过多。dcSSc 的平均红细胞压积为 23.8%,lcSSc 为 29%。

结论

dcSSc 与 GAVE 的更早发生以及需要更多治疗干预的更严重贫血相关。皮肤疾病的快速进展可能提示 GAVE 的更早发生。缺乏抗拓扑异构酶 I 抗体和存在抗 RNA 聚合酶 III/斑点型抗核抗体模式的抗体可能有助于识别 SSc 患者中发生 GAVE 风险增加的亚组。

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