WordNet

an impairment of health or a condition of abnormal functioning
caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological

PrepTutorEJDIC

(体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
女性の話術芸人 =diseur
病気にかかった / 病的な,不健全な(morbid)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/04 13:13:34」(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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1. ブドウ膜炎：病因、臨床症状、および診断 uveitis etiology clinical manifestations and diagnosis
2. ブドウ膜炎：治療 uveitis treatment
3. 様々な抗核抗体 miscellaneous antinuclear antibodies
4. 感染症を繰り返す成人に対するアプローチ approach to the adult with recurrent infections
5. 結核と眼 tuberculosis and the eye

English Journal

Clinical and multimodal imaging characteristics of acute Vogt-Koyanagi-Harada disease unassociated with clinically evident exudative retinal detachment.

Attia S1, Khochtali S, Kahloun R, Ammous D, Jelliti B, Ben Yahia S, Zaouali S, Khairallah M.
International ophthalmology.Int Ophthalmol.2015 May 5. [Epub ahead of print]
The purpose of this study was to describe the clinical and multimodal imaging findings in acute Vogt-Koyanagi-Harada (VKH) disease without clinically evident exudative retinal detachment (ERD). We retrospectively reviewed the charts of 18 patients (36 eyes), diagnosed with acute VKH disease without
PMID 25939987

High rate of clinical recurrence in patients with Vogt-Koyanagi-Harada disease treated with early high-dose corticosteroids.

Sakata VM1, da Silva FT, Hirata CE, Marin ML, Rodrigues H, Kalil J, Costa RA, Yamamoto JH.
Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv für klinische und experimentelle Ophthalmologie.Graefes Arch Clin Exp Ophthalmol.2015 May;253(5):785-90. doi: 10.1007/s00417-014-2904-z. Epub 2015 Jan 16.
PURPOSE: To analyse the rate of clinical recurrences in Brazilian patients with Vogt-Koyanagi-Harada (VKH) disease after early high-dose corticosteroid treatment.METHODS: Retrospective study including patients treated with early high-dose corticosteroids (prednisone, 1-1.5 mg/kg/day, or 3-day 1 g
PMID 25592477

Incidence and clinical features of recurrent Vogt-Koyanagi-Harada disease in Japanese individuals.

Iwahashi C1, Okuno K, Hashida N, Nakai K, Ohguro N, Nishida K.
Japanese journal of ophthalmology.Jpn J Ophthalmol.2015 May;59(3):157-63. doi: 10.1007/s10384-015-0377-1. Epub 2015 Mar 26.
PURPOSE: Our aim was to determine the recurrence rate of inflammation in Vogt-Koyanagi-Harada (VKH) disease and to describe its clinical features.METHODS: We retrospectively evaluated patients diagnosed as having VKH disease with exudative retinal detachment at the Osaka University Hospital or the J
PMID 25808016

Japanese Journal

CLINICAL INVESTIGATION : Differences in the clinical features of two types of Vogt-Koyanagi-Harada disease : serous retinal detachment and optic disc swelling

Okunuki Yoko,Tsubota Kinya,Kezuka Takeshi [他]
Japanese journal of ophthalmology : the official international journal of the Japanese Ophthalmological Society 59(2), 103-108, 2015-03
NAID 40020401099

私の経験原田病の再発時に生じた片眼性前部虚血性視神経症の1例

伊藤愉一胤,長澤真奈,橋爪公平 [他]
眼科 57(1), 77-82, 2015-01
NAID 40020325951

何が見える? 何がわかる? OCT : Series 2 応用編(第22回)苦手なぶどう膜炎はどうみる?

坪井孝太郎,中井慶
臨床眼科 68(12), 1588-1593, 2014-11
NAID 40020243426

Related Pictures

★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	「disease」「Harada disease」

「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

[show details]

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

[show details]

診断

急性両眼整備慢性ぶどう膜炎、蛍光眼底検査による限局性網膜剥離、漿液性髄膜炎

治療

ステロイドパルス療法、あるいはステロイド大量療法が行われる。ステロイド減量時に炎症が再発しうるので、徐々に減少するようにする。
ステロイドの減量がうまくいかない場合、免疫抑制剤の投与が考慮される。

予後

ステロイドによる治療で予後良好であるが、治療が適切でない場合再発を繰り返す遷延型に移行

参考

1.

http://www.fujitaec.or.jp/budoumakuen/budoumakuen_003.html

2. 写真 - sunset glow fundus

http://www.ojhas.org/issue38/2011-2-24.htm

http://www.ojhas.org/issue38/2011-2-24-4.gif

国試

104I071

「ぶどう膜髄膜脳炎症候群」

　　[★]

英: uveomeningoencephalitic syndrome
関: フォークト・小柳・原田症候群フォークト・小柳・原田病 ;関:Vogt-Koyanagi-Harada disease、Vogt-Koyanagi-Harada syndrome

メラノサイトに対する自己免疫疾患

「disease」

　　[★]

n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

something that is very wrong with people's attitudes, way of life or with society.

関: ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

注意

disease ≠ illness ≠ disorder

「Harada disease」

　　[★] 原田病、フォークト・小柳・原田症候群

[1] A Vogt. Frühzeitiges Ergrauen der Zilien und Bemerkungen über den sogenannten plötzlichen Eintritt dieser Veränderung. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1906, 44: 228-242.

[2] Y. Koyanagi. Dysakusis, Alopecie und Poliosis bei schwerer Uveitis nicht traumatischen Ursprungs. Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1929, 82: 194-211.

[3] E. Harada. Clinical study of nonsuppurative choroiditis. A report of acute diffuse choroiditis. Acta Societatis ophthalmologicae Japonicae, 1926, 30: 356.

[4] 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.

匿名

検索

案内

案内

Vogt-Koyanagi-Harada disease