Vogt-小柳-原田症候群のIgA腎症患者の一例

A case presentation of an IgA nephropathy patient with Vogt-Koyanagi-Harada syndrome.

• Quan Zhang
• Xing Fan
• Meng Tian
• Hongling Han

抄録

背景:

Vogt-小柳-原田症候群は、皮膚や睫毛の脱色、慢性肉芽腫性虹彩炎や滲出性網膜剥離、無菌性髄膜炎や脳症を特徴とする稀な疾患です。Vogt-小柳-原田症候群を合併したIgA腎症は、類似の病態を有する可能性はあるが、非常にまれである。IgA腎症と診断された場合、眼球病変は検査されないことが多く、予後に影響を及ぼす。

BACKGROUND: Vogt-Koyanagi-Harada syndrome is a rare disease characterized by skin and eyelash bleaching, chronic granulomatous iridocyclitis and exudative retinal detachment, and aseptic meningitis and encephalopathy. IgA nephropathy complicated by Vogt-Koyanagi-Harada syndrome is very rare, even though they might have similar pathogeneses. Ocular lesions often are not examined when patients are diagnosed with IgA nephropathy, which affects the prognosis.

ケースプレゼンテーション:

高熱と血尿を訴えて入院した55歳の男性IgA腎症患者を紹介する。身体所見は，両眼視機能障害によるぼやけ，聴力障害，胸部と背部にうっ血性発疹を認めた．腎超音波検査では異常なし．臨床検査では糸球体腎炎は感染症を合併しており，抗感染症治療は無効であった．両側フルオレセイン血管造影でVogt-小柳-原田症候群と診断された．腎生検ではIgA腎症が確認された．ホルモンショック療法とシクロホスファミド補助療法を行い，症状は改善した．

CASE PRESENTATION: We describe a 55-year-old male IgA nephropathy patient who was admitted with high fever and hematuria. Physical examination revealed impaired binocular vision with blurred vision, impaired hearing, and a congestive rash on the chest and back. Renal ultrasound examination showed no abnormalities. Laboratory examination showed that glomerulonephritis was complicated by infection, and anti-infection therapy was ineffective. Bilateral fluorescein angiography showed Vogt-Koyanagi-Harada syndrome. Further renal biopsy confirmed IgA nephropathy. Hormone shock therapy and cyclophosphamide adjuvant therapy were administered, and the patient's symptoms improved.

結論:

IgA腎症とVogt-小柳-原田症候群を同時に発症した非常に稀な症例を初めて報告した。Vogt-小柳-原田症候群は発症が早く重篤であるのに対し、IgA腎症は比較的軽度であるため、専門医が軽視しやすい疾患である。特にIgA腎症患者の神経学的異常や眼症状を呈した場合には、免疫抑制療法を適時に行うことで眼疾患の転帰が改善される可能性があるため、臨床的には類似の機序を持つ両疾患の併発に注意を払う必要がある。

CONCLUSION: For the first time, we reported the case of simultaneous onset of IgA nephropathy and Vogt-Koyanagi-Harada syndrome, which is very rare. The onset of Vogt-Koyanagi-Harada syndrome is rapid and serious, while that of IgA nephropathy is relatively milder, making it easy for specialized doctors to neglect this condition. Doctors should be highly alert to the clinical concomitant occurrence of the two diseases with similar mechanisms, especially in the case of neurological defects and ocular symptoms in IgA nephropathy patients, since timely immunosuppressive treatment may improve the outcome of ocular diseases.