Inomata H, Minei M, Taniguchi Y, Nishimura F
Jpn J Ophthalmol. 1983;27(1):9-26.
The eyes in a case of Vogt-Koyanagi-Harada disease (VKH) with long-standing uveitis for 26 years after the onset were studied histopathologically. It was found that typical granulomatous inflammation was persistent in the uveal tract and the choroidal neovascularization occurred in the peripheral fundus accompanied by proliferation of the retinal pigment epithelial cells (RPE). Some of the new vessels under the pigment epithelium extended into the vitreous. It was concluded that the ocular inflammation of VKH was essentially granulomatous even in this long-standing case. Disappearance of choroidal melanocytes, existence of epithelioid cells containing pigment granules, and accumulation of lymphocytes and plasma cells in the lesion indicated that the inflammation was an autoimmune reaction against uveal melanocytes, although the trigger initiating the disease remains unknown. It was further concluded that the peripheral fundus as well as the peripapillary and macular areas was a predilected site for choroidal neovascularization in chronic uveitis. The choroidal neovascularization may develop in such a way that the uveal inflammation damages the Bruch's membrane and choriocapillaris and consequently causes retinal ischemia, thus stimulating the endothelium of the choriocapillaris and the overlying RPE to proliferate. There is a close relation between choroidal neovascularization and proliferation of RPE. Choroidal neovascularization may cause reactive proliferation of the RPE and vice versa.
对1例伏格特-小柳-原田病（VKH）患者发病26年后长期葡萄膜炎的眼部进行了组织病理学研究。发现葡萄膜中典型的肉芽肿性炎症持续存在，周边眼底出现脉络膜新生血管，并伴有视网膜色素上皮细胞（RPE）增殖。色素上皮下的一些新生血管延伸至玻璃体。得出结论，即使在这种病程较长的病例中，VKH的眼部炎症本质上仍是肉芽肿性的。脉络膜黑素细胞消失、含色素颗粒的上皮样细胞存在以及病变中淋巴细胞和浆细胞积聚表明，尽管引发疾病的触发因素尚不清楚，但该炎症是针对葡萄膜黑素细胞的自身免疫反应。进一步得出结论，慢性葡萄膜炎中，周边眼底以及视乳头周围和黄斑区是脉络膜新生血管的好发部位。脉络膜新生血管可能以这样的方式发展，即葡萄膜炎症损害 Bruch 膜和脉络膜毛细血管，进而导致视网膜缺血，从而刺激脉络膜毛细血管内皮和上方的RPE增殖。脉络膜新生血管与RPE增殖之间存在密切关系。脉络膜新生血管可能导致RPE的反应性增殖，反之亦然。
