Bonfioli Adriana A, Orefice Fernando
Eye & Ear Institute of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Semin Ophthalmol. 2005 Jul-Sep;20(3):177-82. doi: 10.1080/08820530500231938.
Sarcoidosis is a multisystem granulomatous disease with an unknown etiology, characterized by the presence of noncaseating granulomas in involved organs. It has a worldwide prevalence, but variable incidence among different geographical regions. The disease affects adults between 20 and 40 years of age, and it is slightly more common in women than men. Sarcoidosis is 3 to 4 times more prevalent in US blacks than whites. It usually presents with bilateral hilar adenopathy, pulmonary infiltrates and skin or eye involvement. The eye or adnexa are affected in 25 to 80% of the sarcoidosis patients. The disease can involve the orbit, lacrimal gland, anterior and posterior segments of the eye. Typical sarcoid uveitis presents with bilateral mutton-fat keratic precipitates, cells, flare, iris nodules, anterior and posterior synechia, and increased ocular pressure. Posterior involvement includes vitreitis, vasculitis, choroidal lesions, and optic neuropathy. Long term complications are common, and cystoid macular edema is the most important and sight-threatening consequence. Laboratory tests for the diagnosis of sarcoidosis include chest radiography or CT scan, bronchoscopy with bronchoalveolar lavage, angiotensin converting enzyme (ACE), Lysozyme, serum and urinary calcium, gallium scintigraphy, and biopsy. The only confirmatory test is biopsy showing classic noncaseating granulomas. Oral corticosteroids are the mainstay of treatment of sarcoidosis. Systemic cytotoxic agents like methrotrexate, azathioprine, and chlorambucil may be used in refractory cases. The visual prognosis of sarcoidosis is usually good.
结节病是一种病因不明的多系统肉芽肿性疾病,其特征是受累器官中存在非干酪样肉芽肿。它在全球范围内都有流行,但在不同地理区域的发病率有所不同。该疾病影响20至40岁的成年人,女性比男性略为常见。在美国,黑人患结节病的患病率是白人的3至4倍。它通常表现为双侧肺门淋巴结肿大、肺部浸润以及皮肤或眼部受累。25%至80%的结节病患者眼部或附属器会受到影响。该疾病可累及眼眶、泪腺、眼球的前段和后段。典型的结节病性葡萄膜炎表现为双侧羊脂状角膜后沉着物、细胞、闪光、虹膜结节、前后粘连以及眼压升高。后部受累包括玻璃体炎、血管炎、脉络膜病变和视神经病变。长期并发症很常见,黄斑囊样水肿是最重要且威胁视力的后果。用于诊断结节病的实验室检查包括胸部X线摄影或CT扫描、支气管镜检查及支气管肺泡灌洗、血管紧张素转换酶(ACE)、溶菌酶、血清和尿钙、镓闪烁显像以及活检。唯一的确诊检查是活检显示典型的非干酪样肉芽肿。口服糖皮质激素是结节病治疗的主要手段。对于难治性病例,可使用甲氨蝶呤、硫唑嘌呤和苯丁酸氮芥等全身性细胞毒性药物。结节病的视力预后通常良好。