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a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/30 23:23:40」(JST)

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Dermatologic manifestation of VKH

Vogt–Koyanagi–Harada syndrome (VKH syndrome) is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous uveitis with accompanying dermatologic, neurologic, and auditory involvement. VKH syndrome is named for ophthalmologists Alfred Vogt from Switzerland and Yoshizo Koyanagi and Einosuke Harada from Japan.^[1]^[2]^[3]

Pathophysiology

VKH syndrome is an immune-mediated disease. The mechanism of the disease is thought to be T helper cell mediated autoimmune attack of melanocytes in the skin, uvea, central nervous system and inner ear. A new T-cell subset Th17 may play an important role in the initiation and maintenance of inflammatory disease when stimulated by the interleukin IL-23, thus producing IL-17.^[4]

There is a higher rate of VKH syndrome in people of Asian, Latin, and Mediterranean descent

Clinical presentation

Uveitis with poliosis of the eyelashes.

There are 4 stages of VKH syndrome: prodromal, acute uveitic, convalescent, and chronic recurrent.

The prodromal stage is marked by flu-like symptoms. Patients present with headache, nausea, meningism, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard) and tinnitus.
The acute uveitic stage is heralded by the onset of blurring of vision in both eyes and is marked by bilateral granulomatous anterior uveitis, variable degree of vitritis, thickening of the posterior choroid with elevation of the peripapillary retinal choroidal layer, hyperemia and edema of the optic nerve, and multiple serous retinal detachments.
The convalescent stage occurs several weeks later and is marked by gradual depigmentation of the choroid, resulting in the classic orange-red discoloration, or sunset-glow fundus appearance. Skin changes including alopecia, vitiligo, and poliosis typically appear during this stage.
The chronic recurrent stage is marked by repeated bouts of uveitis.

Diagnosis

The diagnosis of VKH syndrome is made by ophthalmologist or optometrist based on clinical presentation. Imaging techniques such as fluorescein angiography are used to confirm diagnosis.

Treatment

The acute stage of VKH syndrome is exquisitely responsive to early and aggressive treatment with topical, periocular and systemic corticosteroids and cycloplegic agents.

References

^ A Vogt. Frühzeitiges Ergrauen der Zilien und Bemerkungen über den sogenannten plötzlichen Eintritt dieser Veränderung. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1906, 44: 228-242.
^ Y. Koyanagi. Dysakusis, Alopecie und Poliosis bei schwerer Uveitis nicht traumatischen Ursprungs. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1929, 82: 194-211.
^ E. Harada. Clinical study of nonsuppurative choroiditis. A report of acute diffuse choroiditis. Acta Societatis ophthalmologicae Japonicae, 1926, 30: 356.
^ Bordaberry, MF (Nov 2010). "Vogt-Koyanagi-Harada disease: diagnosis and treatments update.". Current opinion in ophthalmology 21 (6): 430–5. doi:10.1097/ICU.0b013e32833eb78c. PMID 20829689.

External links

VKH Story

UpToDate Contents

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English Journal

JAK1, but not JAK2 and STAT3, confers susceptibility to Vogt-Koyanagi-Harada (VKH) syndrome in a Han Chinese population.

Hu K, Hou S, Li F, Xiang Q, Kijlstra A, Yang P.SourceChongqing Key Laboratory of Ophthalmology, The First Affiliated Hospital of Chongqing Medical University, Chongqing Eye Institute, Chongqing, China.
Investigative ophthalmology & visual science.Invest Ophthalmol Vis Sci.2013 Apr 23. pii: iovs.13-11615v1. doi: 10.1167/iovs.13-11615. [Epub ahead of print]
PURPOSE: Janus kinase 1 (JAK1), JAK2 and signal transducer and activator of transcription 3 (STAT3) play an important role in Th1 and Th17 differentiation and gene polymorphisms of these factors have been demonstrated to be associated with certain autoimmune diseases. The present study was performed
PMID 23611997

Vogt-Koyanagi-Harada syndrome.

Greco A, Fusconi M, Gallo A, Turchetta R, Marinelli C, Macri GF, De Virgilio A, de Vincentiis M.SourceDepartment Organs of Sense, ENT Section, University of Rome "La Sapienza", Italy.
Autoimmunity reviews.Autoimmun Rev.2013 Apr 6. pii: S1568-9972(13)00037-2. doi: 10.1016/j.autrev.2013.01.004. [Epub ahead of print]
OBJECTIVES: The objectives of this study are to review our current knowledge of the aetiopathogenesis of Vogt-Koyanagi-Harada syndrome, including viral infection, genetic factors and immunomediated mechanisms, and to discuss pathogenesis and its relevance to pharmacotherapy.SYSTEMATIC REVIEW METHODO
PMID 23567866

The outcomes of indocyanine green angiography monitored immunotherapy in Vogt-Koyanagi-Harada disease.

Chee SP, Jap A.SourceOcular Inflammation and Immunology Service, Singapore National Eye Centre, 11 Third Hospital Avenue, Singapore 168751, Singapore. chee.soon.phaik@snec.com.sg
The British journal of ophthalmology.Br J Ophthalmol.2013 Feb;97(2):130-3. doi: 10.1136/bjophthalmol-2012-302538. Epub 2012 Dec 4.
AIMS: To compare outcomes of indocyanine green angiography (ICGA) versus clinically monitored immunotherapy in Vogt-Koyanagi-Harada (VKH) disease.METHODS: Consecutive patients of Singapore National Eye Centre with VKH receiving high-dose corticosteroids within 4 weeks of onset of symptoms had therap
PMID 23212203

Japanese Journal

Usefulness of Anterior Chamber Depth Measurement for Efficacy Assessment of Steroid Pulse Therapy in Patients with Vogt-Koyanagi-Harada Disease

OTSUKI Tomohiro,SHIMIZU Kimiya,IGARASHI Akihito,KAMIYA Kazutaka
Japanese journal of ophthalmology 54(5), 396-400, 2010-09-01
NAID 10027665648

原田病・掌蹠膿疱症・橋本病の3つの自己免疫疾患に新規発症の1型糖尿病を合併した1例

西真貴子,大橋健,大須賀淳一,塚本和久,植木浩二郎,門脇孝
日本内科学会雑誌 98(6), 1369-1371, 2009-06-10
NAID 10024935160

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★リンクテーブル★

リンク元	「フォークト・小柳・原田病」「ぶどう膜髄膜脳炎症候群」
関連記事	「syndrome」

「フォークト・小柳・原田病」

　　[★]

英: Vogt-Koyanagi-Harada disease
同: フォークト-小柳-原田症候群 Vogt-Koyanagi-Harada syndrome、Vogt-Koyanagi-Harada病、ぶどう膜髄膜脳炎症候群ぶどう膜・髄膜・脳炎症候群 uveo-meningo-encephalitis syndrome、特発性ぶどう膜炎 idiopathic uveitis、原田病 Harada disease、小柳病 Koyanagi disease
関: 交感性眼炎、ぶどう膜炎

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フォークト・小柳・原田症候群 : 約 32,700 件
フォークト・小柳・原田病 : 約 49,500 件
Vogt-Koyanagi-Harada病 : 78 件
Vogt-Koyanagi-Harada症候群 : 14 件
Vogt-Koyanagi-Harada disease : 約 1,820 件
Vogt-Koyanagi-Harada syndrome : 約 1,850 件

en

Vogt-Koyanagi-Harada disease : 約 18,100 件
Vogt-Koyanagi-Harada syndrome : 約 97,900 件

概念

全身のメラノサイトを標的とする原因不明の自己免疫疾患。

疫学

有病率：人口100万人対15.5人(SOP.57)
罹患率：人口100万人対6.3人(SOP.57)

遺伝

日本人を中心としたアジア人に多発
HLA-B54(35%)、HLA-DR4(95%)、HLA-DR53(100%)、HLA-DQ4(90%)　(SOP.57)　←　患者におけるHLAのタイピング？

病態と症候

症状は全て色素細胞(髄膜、ぶどう膜、内耳、毛髪)を中心とした炎症の結果生じる

眼　：両眼性急性汎ぶどう膜炎、虹彩毛様体炎、脈絡膜炎による漿液性網膜剥離(剥離部は脱色素を起こして夕焼け状眼底を引き起こす)。回復期に夕焼け状眼底(脈絡膜の色素脱出による赤みを帯びた眼底)、角膜輪部分色素消失

全身：漿液性髄膜炎、内耳症状(難聴、耳鳴り、めまい)、皮膚症状(皮膚白斑)、毛髪症状(白髪、脱毛)

症状

視力低下(網膜下の漿液が吸収され玲玲場視力は回復する)

合併症

遷延した虹彩毛様体炎、併発白内障、併発緑内障

検査

髄液検査：小リンパ球主体の増加
細隙灯顕微鏡
蛍光眼底検査：(炎症の活動期(眼病気))脈絡膜からの多発性点状蛍光漏出