Orbital and Adnexal Involvement in Sarcoidosis: Analysis of Clinical Features and Systemic Disease In 30 Cases

Orbital and Adnexal Involvement in Sarcoidosis: Analysis of Clinical Features and Systemic Disease In 30 Cases
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DOI:
10.1016/j.ajo.2010.12.011
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发表时间:
2011-06-01
影响因子:
4.2
通讯作者:
Christianson, Murray D.
Christianson, Murray D.
中科院分区:
医学1区
文献类型:
--
作者:
Demirci, Hakan;Christianson, Murray D.

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目的:评价经活检证实的眼眶和附件结节病的临床特征、治疗和预后。设计:观察性、回顾性病例系列。方法:回顾性分析1个中心连续收治的30例患者的临床特点和治疗方法。使用单变量和多变量回归模型分析11例无全身性疾病患者的数据对全身性结节病发生的影响。结果:眼眶及附件受累包括泪腺占63%(19/30),眼睑占17%(5/30),眼眶占13%(4/30),泪囊占7%(2/30)。37%(11/30)患者已知有全身性疾病。在只有眼眶病变的63%(19/30)病例中,最初发现全身性病变的占27%(8/30),随后发现全身性病变的占7%(2/30),30%(9/30)未发现全身性病变。在计算机断层扫描中,85%(25/30)的病变界限分明,15%(5/30)的病变弥漫性。治疗方法包括50%(15/30)的有症状疾病患者接受全身类固醇治疗,27%(8/30)的患者接受手术切除,23%(7/30)的无症状疾病患者接受观察。平均随访44个月后,所有病例均出现消退或保持稳定,但接受全身类固醇治疗的病例中有7%(1/30)复发。使用Kaplan-Meier估计,系统性结节病在5年内的发生率为8%。在单因素或多因素分析中,没有发现任何临床特征可以显著预测系统性结节病。结论:在我们的研究中,仅患有眼眶和附件结节病的患者,在5年内发生全身性疾病的比例为8%。全身性类固醇对有症状性疾病的患者有效。[J]中华眼科杂志,2011;51(1):1074-1080。(C)爱思唯尔公司2011。版权所有。)
PURPOSE: To evaluate clinical features, management, and prognosis of biopsy-proven orbital and adnexal sarcoidosis.DESIGN: Observational, retrospective case series.METHODS: Clinical features and treatment methods were evaluated retrospectively in 30 consecutive patients managed in 1 center. Data from 11 patients without systemic disease at presentation were analyzed for their impact on occurrence of systemic sarcoidosis using univariate and multivariate regression models.RESULTS: Orbital and adnexal involvement included the lacrimal gland in 63% (19/30), the eyelid in 17% (5/30), the orbit in 13% (4/30), and the lacrimal sac in 7% (2/30). Thirty-seven percent (11/30) had known systemic disease. Of 63% (19/30) of cases with only orbital disease, systemic disease initially was found in 27% (8/30), subsequently in 7% (2/30), and never in 30% (9/30). On computed tomography scans, the lesions were well circumscribed in 85% (25/30) and diffuse in 15% (5/30). Management included systemic steroids in 50% (15/30) of cases with symptomatic disease, followed by excision in 27% (8/30) of cases and observation in 23% (7/30) of cases with asymptomatic disease. After a mean follow-up of 44 months, all cases showed regression or remained stable, but 7% (1/30) of cases treated with systemic steroids demonstrated recurrence. Using Kaplan-Meier estimates, systemic sarcoidosis developed in 8% at 5 years. In univariate or multivariate analysis, no clinical feature was found to be significantly predictive of systemic sarcoidosis.CONCLUSIONS: In our series, in patients with orbital and adnexal sarcoidosis alone, systemic disease developed in 8% by 5 years. Systemic steroids were effective for patients with symptomatic disease. (Am J Ophthalmol 2011;151:1074-1080. (C) 2011 by Elsevier Inc. All rights reserved.)