Tarsal-conjunctival disease associated with Wegener's granulomatosis.
Tarsal-conjunctival disease associated with Wegener's granulomatosis.
复制标题
与韦格纳肉芽肿病相关的睑板结膜疾病。
DOI：
10.1016/s0161-6420(03)00616-x
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发表时间：
2003
期刊：
影响因子：
13.7
通讯作者：
Smith,JanineA
中科院分区：
文献类型：
--
作者：
Robinson,MichaelR;Lee,SusanS;Sneller,MichaelC;Lerner,Ruby;Langford,CarolA;Talar-Williams,Cheryl;Cox,TerryA;Chan,ChiC;Smith,JanineA
目的描述韦格纳肉芽肿病(WG)患者的临床特点。设计：病例对照研究。对1996年1月至2002年6月在美国国立卫生研究院接受眼科检查的82例WG患者的临床资料进行回顾分析。方法记录眼科检查、内科治疗结果及组织病理学分析结果。睑板结膜病的定义是：(1)结膜充血和肉芽肿形成，坏死区，或眼睑或结膜中活跃的纤维血管改变，或(2)不活跃的纤维血管疤痕。我们用贝叶斯方法评估了踝结膜疾病与主要器官系统受累的关系。主要结果测量在一组WG患者中，踝结膜疾病的发生和临床特征，以及与主要器官系统受累的关系。结果在6.5年期间接受WG检查的82例患者中，13例(16%)发生了踝结膜疾病。最常累及上眼睑表面，表现为结膜充血7例，肉芽肿性病变3例，睑板结膜坏死4例，纤维血管增生活跃6例，纤维瘢痕组织不活跃7例。眼睑活检标本的组织病理学分析显示，眼睑和结膜有肉芽肿性炎症、局灶性坏死和闭塞性脉管炎。在回顾合并和不合并睑板结膜病患者的器官受累模式时，声门下狭窄和鼻泪管阻塞与睑板结膜病之间的关系显示出很高的临床意义。结论幕上结膜病是以前在WG患者中不常见的发现，其特征是眼睑结膜和睑板的炎症，继而纤维血管增殖和瘢痕形成。由于睑结膜疾病与声门下狭窄密切相关，可导致喉部阻塞和呼吸衰竭，患者应转诊至耳鼻咽喉科医生进行评估。
OBJECTIVETo describe the clinical characteristics of tarsal–conjunctival disease in a cohort of patients with Wegener's granulomatosis (WG).DESIGNRetrospective, case-controlled study.PARTICIPANTSThe medical records of 82 consecutive WG patients who underwent an eye examination between January 1996 and June 2002 at the National Institutes of Health were reviewed.METHODSDetails of the ophthalmic examination, results of medical therapy, and histopathologic analysis results were recorded. Tarsal–conjunctival disease was defined by (1) conjunctival hyperemia and granuloma formation, areas of necrosis, or active fibrovascular changes in the tarsus or conjunctiva, or (2) evidence of inactive fibrovascular scar. The association of tarsal–conjunctival disease with major organ system involvement was assessed using Bayesian methods.MAIN OUTCOME MEASURESThe occurrence and clinical characteristics of tarsal–conjunctival disease in a cohort of patients with WG and associations with major organ system involvement.RESULTSTarsal–conjunctival disease occurred in 13 of 82 patients (16%) with WG examined over a 6.5-year period. The palpebral surface of the upper lid was involved most commonly, showing conjunctival hyperemia in seven patients, granulomatous lesions in three patients, tarsal–conjunctival necrosis in four patients, active fibrovascular proliferation in six patients, and inactive fibrous scar tissue in seven patients. Histopathologic analysis of eyelid biopsy specimens showed granulomatous inflammation, focal necrosis, and areas of occlusive vasculitis in the tarsus and conjunctiva. In reviewing the patterns of organ involvement in patients with and without tarsal–conjunctival disease, the association of subglottic stenosis and nasolacrimal duct obstruction with tarsal–conjunctival disease showed a high probability of clinical significance.CONCLUSIONSTarsal–conjunctival disease, a previously uncommon finding in patients with WG, was characterized by inflammation of the palpebral conjunctiva and tarsus followed by a fibrovascular proliferation and scar formation. Because of the important association of tarsal–conjunctival disease with subglottic stenosis, which can progress and lead to laryngeal obstruction and respiratory failure, patients with tarsal–conjunctival disease should be referred to an otolaryngologist for evaluation.