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付格特-小柳-原田综合征(VKHS)是一种主要累及视觉、听觉、脑膜和皮肤的自身免疫性炎性疾病。其靶目标是选择性侵犯抗原相关黑色素细胞。一般而言,VKHS的症状以听觉和神经系统的症状为多见,但多继发于视乳头水肿和视网膜剥离等所致的视力下降之后。VKHS患者神经系统的表现可与急性脑膜脑炎相似,脑脊液亦可表现为多形核白细胞的增多,故需与感染性脑膜脑炎相鉴别。总之,VKHS是一个累及多个系统的自身免疫疾病。发病机制未明,临床表现多样,漏诊、误诊率高。其中枢神经系统表现可与脑膜炎相似。临床上,当患者出现急性视物不清、耳鸣、感觉神经性耳聋、白癫风、秃发和脑膜炎等症状和(或)体征时,应考虑到VKHS的可能。并尽早行大剂量激素冲击治疗以缩短病程、改善预后和减少并发症的发生。
Fuget - Koyanagi - Harada syndrome (VKHS) is a mainly involving the visual, auditory, meningeal and skin autoimmune inflammatory diseases. Its target is selective invasion of antigen-associated melanocytes. In general, the symptoms of VKHS are more common in auditory and neurological conditions, but are often secondary to decreased visual acuity due to papilledema and retinal detachment. VKHS patients with neurological manifestations may be similar to acute meningoencephalitis, cerebrospinal fluid can also be manifested as an increase in polymorphonuclear leukocytes, it should be differentiated from infectious meningoencephalitis. In conclusion, VKHS is an autoimmune disease that affects multiple systems. The pathogenesis is unknown, the clinical manifestations are diverse, missed diagnosis, misdiagnosis rate is high. Its central nervous system performance may be similar to meningitis. Clinically, VKHS should be considered when patients have symptoms and / or signs of acute visual impairment, tinnitus, sensory nerve deafness, vitiligo, alopecia, and meningitis. And high-dose hormonal impact treatment as soon as possible to shorten the course of the disease, improve prognosis and reduce the incidence of complications.