Vogt-Koyanagi-Harada disease in a patient with retinitis pigmentosa in one eye and pigmented paravenous retinochoroidal atrophy in the other eye

Q3 Medicine
Junya Sato, Kouhei Hashizume, Yasuhiro Nishida, Shigenori Miyoshi, Mana Nagasawa, Daijiro Kurosaka
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Abstract

Purpose

To report a case of Vogt-Koyanagi-Harada (VKH) disease in a patient with retinitis pigmentosa (RP) in the right eye and pigmented paravenous retinochoroidal atrophy (PPRCA) in the left eye.

Observations

A 32-year-old woman with a history of RP visited our hospital with blurred vision in the left eye. She had a headache for four days before the onset of vision loss. Slit-lamp examination revealed bilateral inflammation of the anterior chamber and vitreous. Fundus examination revealed atrophy of the retinal pigment epithelium (RPE) and diffuse bone spicule pigmentation in the right eye, as well as retinochoroidal atrophy in the peripapillary region and extending along the retinal veins, accompanied by bone spicule pigmentation in the left eye. Optical coherence tomography (OCT) demonstrated serous retinal detachment (SRD) with hyperreflective material only in the left eye and increased choroidal thickness (CT) around the fovea in both eyes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis. The patient was diagnosed with incomplete VKH disease. After treatment with methylprednisolone, SRD in the left eye completely disappeared, and CT decreased bilaterally.

Conclusions and Importance

SRD, one of the major characteristic symptoms of VKH disease, may not be common in patients with both RP and VKH disease. VKH disease is associated with meningitis, vitiligo, and hearing loss, but signs other than meningitis are less frequent. However, in RP patients with uveitis, examination of the patient for these non-ocular signs may be needed to diagnose VKH disease.
Vogt-Koyanagi-Harada病患者单眼色素性视网膜炎,另眼色素性静脉视网膜脉络膜萎缩
目的报告1例右眼色素性视网膜炎(RP)和左眼色素性静脉视网膜脉络膜萎缩(PPRCA)患者发生Vogt-Koyanagi-Harada (VKH)病。患者32岁,女,有RP病史,左眼视力模糊。在视力丧失之前,她头痛了四天。裂隙灯检查显示双侧前房及玻璃体炎症。眼底检查显示右眼视网膜色素上皮(RPE)萎缩,弥漫性骨针状色素沉着,乳头周围区视网膜脉络膜萎缩,沿视网膜静脉延伸,左眼伴有骨针状色素沉着。光学相干断层扫描(OCT)显示浆液性视网膜脱离(SRD),仅在左眼有高反射物质,双眼中央窝周围的脉络膜厚度(CT)增加。脑脊液分析显示淋巴细胞增多症。患者被诊断为不完全VKH病。经甲强的松龙治疗后,左眼SRD完全消失,双侧CT下降。结论及重要性esrd是VKH疾病的主要特征性症状之一,在RP和VKH疾病患者中可能并不常见。VKH病与脑膜炎、白癜风和听力丧失有关,但脑膜炎以外的症状较少见。然而,对于伴有葡萄膜炎的RP患者,可能需要检查患者的这些非眼部体征来诊断VKH疾病。
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来源期刊
CiteScore
2.40
自引率
0.00%
发文量
513
审稿时长
16 weeks
期刊介绍: The American Journal of Ophthalmology Case Reports is a peer-reviewed, scientific publication that welcomes the submission of original, previously unpublished case report manuscripts directed to ophthalmologists and visual science specialists. The cases shall be challenging and stimulating but shall also be presented in an educational format to engage the readers as if they are working alongside with the caring clinician scientists to manage the patients. Submissions shall be clear, concise, and well-documented reports. Brief reports and case series submissions on specific themes are also very welcome.
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