International Criteria for the Diagnosis of Ocular Sarcoidosis: Results of the First International Workshop on Ocular Sarcoidosis (IWOS)
International Criteria for the Diagnosis of Ocular Sarcoidosis: Results of the First International Workshop on Ocular Sarcoidosis (IWOS)
复制标题
DOI：
10.1080/09273940902818861
复制
发表时间：
2009-01-01
影响因子：
3.3
通讯作者：
Mochizuki, Manabu
中科院分区：
文献类型：
--
作者：
Herbort, Carl P.;Rao, Narsing A.;Mochizuki, Manabu
目的：报告眼内结节病的诊断标准，考虑提示性临床体征和适当的实验室检查和活检结果。设计：国际命名委员会共识研讨会。研究方法：2006年10月28-29日，一个来自亚洲、非洲、欧洲和美洲的葡萄膜炎专家国际小组在东京品川举行了一次会议。根据会议前发出的调查问卷，与会者讨论了符合眼结节病诊断标准的潜在眼内临床体征。临床体征的精确定义获得了三分之二的多数票，被纳入与眼部结节病一致的体征列表中。实验室检查也进行了类似的讨论，那些达到三分之二多数的检查被保留用于眼结节病的诊断。最后，根据眼部体征、实验室检查和活检结果提出了诊断标准。结果如下：该共识会议确定了眼内结节病诊断的七个体征：（1）羊脂性角膜沉淀物（KPs）/小肉芽肿性KPs和/或虹膜结节（Koeppe/Busacca），（2）小梁网（TM）结节和/或帐篷状周边前粘连（PAS），（3）玻璃体混浊显示雪球/珍珠串，（4）多发性脉络膜视网膜周边病变（活动性和/或萎缩性），（5）发炎眼睛中的结节性和/或节段性静脉周围炎（+/-蜡滴）和/或视网膜巨噬细胞症，6）视盘结节/肉芽肿和/或孤立性脉络膜结节，和（7）双侧性。在具有上述眼内体征的患者中，被判定为对眼结节病的诊断有价值的实验室检查或检查程序包括：（1）BCG接种患者或先前结核菌素皮肤试验阳性的患者的结核菌素皮肤试验阴性，（2）血清血管紧张素转化酶（ACE）水平升高和/或血清溶菌酶升高，（3）胸部X线检查显示双侧肺门淋巴结病（BHL），（4）肝酶检查异常，（5）胸部X线检查阴性患者的胸部CT扫描。眼部结节病诊断的四个确定性水平在排除了其他可能的葡萄膜炎原因的患者中推荐（诊断标准）：（1）活检支持的诊断与相容性葡萄膜炎标记为明确的眼部结节病;（2）如果没有进行活检，但胸部X线检查显示与相容性葡萄膜炎相关的BHL阳性，则标记为假定的眼部结节病;（3）如果未进行活检，胸部X线检查未显示BHL，但存在上述眼内体征中的3种和2项阳性实验室检查，则该病症被标记为可能的眼部结节病;（4）如果进行了肺活检，结果为阴性，但存在上述体征中的至少4种和2项阳性实验室检查，则该病症被标记为可能的眼部结节病。结论：各种临床体征，实验室检查和活检结果提供了四种诊断类型的结节病葡萄膜炎。该分类允许使用标准化术语进行前瞻性多国临床试验，该术语可作为比较各种治疗方式的视觉结果的平台。
Aim: To report criteria for the diagnosis of intraocular sarcoidosis, taking into account suggestive clinical signs and appropriate laboratory investigations and biopsy results. Design: Concensus workshop of an international committee on nomenclature. Methods: An international group of uveitis specialists from Asia, Africa, Europe, and America met in a concensus conference in Shinagawa, Tokyo on October 28-29, 2006. Based on questionnaires that had been sent out prior to the conference, the participants discussed potential intraocular clinical signs eligible for a diagnosis of ocular sarcoidosis. A refined definition of clinical signs, which received two-thirds majority of votes, was included in the list of signs consistent with ocular sarcoidosis. Laboratory investigations were similarly discussed and those tests reaching a two-thirds majority were retained for the diagnosis of ocular sarcoidosis. Finally diagnostic criteria were proposed based on ocular signs, laboratory investigations, and biopsy results. Results: The concensus conference identified seven signs in the diagnosis of intraocular sarcoidosis: (1) mutton-fat keratic precipitates (KPs)/small granulomatous KPs and/or iris nodules (Koeppe/Busacca), (2) trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS), (3) vitreous opacities displaying snowballs/strings of pearls, (4) multiple chorioretinal peripheral lesions (active and/or atrophic), (5) nodular and/or segmental peri-phlebitis (+/- candlewax drippings) and/or retinal macroaneurism in an inflamed eye, 6) optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule, and (7) bilaterality. The laboratory investigations or investigational procedures that were judged to provide value in the diagnosis of ocular sarcoidosis in patients having the above intraocular signs included (1) negative tuberculin skin test in a BCG-vaccinated patient or in a patient having had a positive tuberculin skin test previously, (2) elevated serum angiotensin converting enzyme (ACE) levels and/or elevated serum lysozyme, (3) chest x-ray revealing bilateral hilar lymphadenopathy (BHL), (4) abnormal liver enzyme tests, and (5) chest CT scan in patients with a negative chest x-ray result. Four levels of certainty for the diagnosis of ocular sarcoidosis (diagnostic criteria) were recommended in patients in whom other possible causes of uveitis had been excluded: (1) biopsy-supported diagnosis with a compatible uveitis was labeled as definite ocular sarcoidosis; (2) if biopsy was not done but chest x-ray was positive showing BHL associated with a compatible uveitis, the condition was labeled as presumed ocular sarcoidosis; (3) if biopsy was not done and the chest x-ray did not show BHL but there were 3 of the above intraocular signs and 2 positive laboratory tests, the condition was labeled as probable ocular sarcoidosis; and (4) if lung biopsy was done and the result was negative but at least 4 of the above signs and 2 positive laboratory investigations were present, the condition was labeled as possible ocular sarcoidosis. Conclusion: Various clinical signs, laboratory investigations, and biopsy results provided four diagnostic categories of sarcoid uveitis. The categorization allows prospective multinational clinical trials to be conducted using a standardized nomenclature, which serves as a platform for comparison of visual outcomes with various therapeutic modalities.