allergic granulomatous angiitis

アレルギー性肉芽腫性血管炎

WordNet

1. characterized by or caused by allergy; "an allergic reaction"
2. having an allergy or peculiar or excessive susceptibility (especially to a specific factor); "allergic children"; "hypersensitive to pollen" (同)hypersensitive, hypersensitized, hypersensitised, sensitized, sensitised, supersensitive, supersensitized, supersensitised
3. relating to or characterized by granulomas
4. inflammation of a blood vessel or lymph duct

PrepTutorEJDIC

1. アレルギー性の;(…に)アレルギーを起こす《+『to』+『名』》 / 《補語にのみ用いて》《話》(…に)神経過敏の,(…が)大きらいの《+『to』+『名』(do『ing)』》

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/02/24 13:56:29」(JST)

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Vasculitis
Classification and external resources
Micrograph showing a vasculitis (Churg-Strauss syndrome). H&E stain.
ICD-10	I77.6, I80, L95, M30-M31
ICD-9	446, 447.6
DiseasesDB	13750
MeSH	D014657

Vasculitis (plural: vasculitides) is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily due to leukocyte migration and resultant damage.

Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) on their own are separate entities.

Classification

Vasculitis can be classified by the cause, the location, the type of vessel or the size of vessel.

• Underlying cause. For example, the cause of syphilitic aortitis is infectious (aortitis simply refers to arteritis of the aorta, which is an artery.) However, the cause of many forms of vasculitis are poorly understood. There is usually an immune component, but the trigger is often not identified. In these cases, the antibody found is sometimes used in classification, as in ANCA-associated vasculitides.
• Location of the affected vessels. For example, ICD-10 classifies "vasculitis limited to skin" with skin conditions (under "L"), and "necrotizing vasculopathies" with musculoskeletal system and connective tissue conditions (under "M"). Arteritis/phlebitis on their own are classified with circulatory conditions (under "I").
• Type or size of the blood vessels that they predominantly affect. Apart from the arteritis/phlebitis distinction mentioned above, vasculitis is often classified by the caliber of the vessel affected. However, there can be some variation in the size of the vessels affected.

According to the size of the vessel affected, vasculitis can be classified into:

• Large vessel: Polymyalgia rheumatica, Takayasu's arteritis, Temporal arteritis
• Medium vessel: Buerger's disease, Cutaneous vasculitis, Kawasaki disease, Polyarteritis nodosa
• Small vessel: Behçet's syndrome, Churg–Strauss syndrome, cutaneous vasculitis, Henoch–Schönlein purpura, Microscopic polyangiitis, Wegener's granulomatosis

Conditions

Some disorders have vasculitis as their main feature. The major types are given in the table below:

Comparison of major types of vasculitis
Vasculitis	Affected organs	Histopathology
Cutaneous small-vessel vasculitis	Skin, kidneys	Neutrophils, fibrinoid necrosis
Wegener's granulomatosis	Nose, lungs, kidneys	Neutrophils, giant cells
Churg–Strauss syndrome	Lungs, kidneys, heart, skin	Histiocytes, eosinophils
Kawasaki disease	Skin, heart, mouth, eyes	Lymphocytes, endothelial necrosis
Buerger's disease	Leg arteries and veins (gangrene)	Neutrophils, granulomas
"Limited" Wegener's vasculitis	Commonly sinuses, brain, and skin; can affect other organs such as lungs, kidneys, joints;

Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis.

Furthermore, there are many conditions that have vasculitis as an accompanying or atypical symptom, including:

• Rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and dermatomyositis
• Cancer, such as lymphomas
• Infections, such as hepatitis C
• Exposure to chemicals and drugs, such as amphetamines, cocaine, and anthrax vaccines which contain the Anthrax Protective Antigen as the primary ingredient.

Symptoms

Petechia and purpura on the low limb due to medication induced vasculitis.

Possible symptoms include:

• General symptoms: Fever, weight loss
• Skin: Palpable purpura, livedo reticularis
• Muscles and joints: Myalgia or myositis, arthralgia or arthritis
• Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss
• Heart and arteries: Myocardial infarction, hypertension, gangrene
• Respiratory tract: Nose bleeds, bloody cough, lung infiltrates
• GI tract: Abdominal pain, bloody stool, perforations
• Kidneys: Glomerulonephritis

Diagnosis

Severe Vasculitis of the major vessels, displayed on FDG-PET/CT

• Laboratory tests of blood or body fluids are performed for patients with active vasculitis. Their results will generally show signs of inflammation in the body, such as increased erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), anemia, increased white blood cell count and eosinophilia. Other possible findings are elevated antineutrophil cytoplasmic antibody (ANCA) levels and hematuria.
• Other organ functional tests may be abnormal. Specific abnormalities depend on the degree of various organs involvement. A Brainspect can show decreased blood flow to the brain and brain damage.
• The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain and kidney. The biopsy elucidates the pattern of blood vessel inflammation.
• An alternative to biopsy can be an angiogram (x-ray test of the blood vessels). It can demonstrate characteristic patterns of inflammation in affected blood vessels.
• Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.

Treatment

Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, cortisone-related medications, such as prednisone, are used. Additionally, other immune suppression drugs, such as cyclophosphamide and others, are considered. In case of an infection, antimicrobial agents including cephalexin may be prescribed. Affected organs (such as the heart or lungs) may require specific medical treatment intended to improve their function during the active phase of the disease.

See also

Sturge–Weber syndrome

References

UpToDate Contents

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• 1. アレルギー疾患リスクのある乳児への粉ミルクおよび固形食の導入 introducing formula and solid foods to infants at risk for allergic disease
• 2. 局所麻酔剤に対するアレルギー反応 allergic reactions to local anesthetics
• 3. 食肉アレルギー allergy to meats
• 4. 非HIV感染患者におけるスルホンアミドアレルギー sulfonamide allergy in non hiv infected patients
• 5. 小児期の食物アレルギーの自然経過 the natural history of childhood food allergy

English Journal

• Association of ANCA-associated vasculitis-rheumatoid arthritis overlap syndrome in four patients: rituximab may be the right choice?

• Szilasi M, Mátyus J, File I, Szücs G, Rákóczi E, Pfliegler G, Szabó Z, Végh E, Szekanecz Z.SourceDepartment of Pulmonology, University of Debrecen Medical and Health Sciences Center, Debrecen, Hungary.
• Autoimmunity.Autoimmunity.2012 Jun;45(4):304-9. Epub 2012 Apr 10.
• OBJECTIVES: There have been few reports on the association of rheumatoid arthritis (RA) with anti-neutrophil cytoplasmic antigen (ANCA)-associated systemic vasculitides.METHODS: Here we present four cases of RA overlapping with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-
• PMID 22443800

• Diagnostics and Therapy of Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitides.

• Reinhold-Keller E.SourceKlinikum Bad Bramstedt & Internistisch-rheumatologische Gemeinschaftspraxis Hamburg, Jürgensallee 44, 22609 Hamburg. reinhold-keller@rheuma-spezialisten.de.
• Current pharmaceutical design.Curr Pharm Des.2012 May 16. [Epub ahead of print]
• Wegener�s granulomatosis (WG), microscopic polyangiitis (MPA) and Churg Strauss syndrome (CSS) are primary systemic vasculitides which predominantly affect small vessels, showing a high association with a positive C/PR3-ANCA in WG and P/MPO-ANCA in MPA, so called ANCA-associated vasculitides (AAV)
• PMID 22612757

Japanese Journal

• カラー臨床報告 前部虚血性視神経症と網膜中心動脈閉塞症が併発したアレルギー性肉芽腫性血管炎(Churg-Strauss症候群)の症例

• 井上 千鶴,中道 悠太,杉山 千晶 [他]
• 臨床眼科 67(3), 369-376, 2013-03
• NAID 40019613797

• アレルギー性肉芽腫性血管炎を合併した Buerger's disease の1例

• 地引 政利,工藤 敏文,光岡 明人,井上 芳徳,三浦 圭子,小林 大輔
• 日本血管外科学会雑誌 = The Japanese journal of vascular surgery : official journal of the Japanese Society for Vascular Surgery 21(5), 691-694, 2012-08-25
• NAID 10031121365

• 難治化病態としてのAGAとABPA (特集 難治性喘息研究の新展開)

• 大林 王司
• 呼吸器内科 21(1), 31-39, 2012-01
• NAID 40019201981

Related Links

• [Allergic granulomatous angiitis].

Allergic granulomatous angiitis (AGA) is a rare disease, which varies in clinical manifestation. The clinical course includes three stages. In the first stage most of the patients show symptoms of respiratory disease, 82 per cent of the patients has ...

• allergic granulomatous angiitis - 日本消化器外科学会

アレルギー性肉芽腫性血管炎（allergic granuloma- tous angiitis ; AGA）は1951年 Churg と Strauss により. 提唱された壊死性血管炎と血管外肉芽腫性病変を特徴. と する疾患で Churg-Strauss 症候群とも呼ばれてい. る1）．臨床症状は気管支喘息， 多発性 ...

Related Pictures

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★リンクテーブル★

リンク元	「アレルギー性肉芽腫性血管炎」「CSS」「AGA」
関連記事	「granulomatous angiitis」「allergic」「granulomatous」

「アレルギー性肉芽腫性血管炎」

　　[★]

英

allergic granulomatous angiitis, AGA

同

チャーグ-ストラウス症候群 チャーグ・ストラウス症候群 Churg-Strauss症候群 Churg-Strauss syndrome CSS、チャーグ・ストラウス血管炎

関

全身性血管炎、MPO-ANCA。難病

• MPO-ANCA(P-ANCA)陽性

概念

• 主に中動脈以下の動脈を好発部位とする壊死性肉芽腫性血管炎
• 高率に肺病変と好酸球増多症を認め、また好酸球の組織浸潤も著明　(⇔古典的多発動脈炎)
• 気管支喘息　＋　好酸球増加　＋　血管炎　→　チャーグ・ストラウス症候群
• チャーグ・ストラウス症候群　＋　典型的組織像　→　アレルギー性肉芽腫性血管炎

症状

主要臨床所見

• 1. 気管支喘息
• 2. 好酸球増加
• 3. 血管炎症候群

症候スペクトル

病態	レイノー現象	抗核抗体	リウマトイド因子	抗好中球細胞質抗体	皮疹	皮下結節	関節炎	筋炎	漿膜炎	自己抗体
										MPO ANCA
病理	壊死性血管炎	糸球体腎炎	間質性肺炎	心炎	唾液腺炎	オニオンスキン病変	ワイヤーループ病変	ヘマトキシリン体	LE細胞

検査

• 血液検査：

• 抗好中球抗体：MPO-ANCA/P-ANCA陽性

アレルギー性肉芽腫性血管炎(Churg-Strauss症候群)の診断基準 (厚生省 難治性血管炎分科会、1998年修正案)

概念

Churg-Straussが古典的PNより分離独立させた血管炎であり気管支喘息、好酸球増加、血管炎による症状を示すものをChurg-Strauss症候群、典型的組織所見を伴うものをアレルギー性肉芽腫性血管炎とする。

診断基準

• 1.主要臨床所見

• (1) 気管支喘息あるいはアレルギー性鼻炎
• (2) 好酸球増加
• (3) 血管炎による症状〔発熱(38℃以上、2週間以上)、体重減少(6か月以内に6kg以上)、多発性単神経炎、消化管出血、紫斑、多関節痛(炎)、筋肉痛、筋力低下〕

• 2.臨床経過の特徴

• 主要所見(1)、(2)が先行し、(3)が発症する。

• 3.主要組織所見

• (1) 周囲組織に著明な好酸球浸潤を伴う細小血管の肉芽腫性、またはフィブリノイド壊死性血管炎の存在
• (2) 血管外肉芽腫の存在

• 4.判定

• (1) 確実(definite)

• (a) 主要臨床所見のうち気管支喘息あるいはアレルギー性鼻炎、好酸球増加および血管炎による症状のそれぞれ一つ以上を示し同時に、主要組織所見の1項目を満たす場合(アレルギー性肉芽腫性血管炎)
• (b) 主要臨床所見3項目を満たし、臨床経過の特徴を示した場合(Churg-Strauss症候群)

• (2) 疑い(probable)

• (a) 主要臨床所見1項目および主要組織所見の1項目を満たす場合(アレルギー性肉芽腫性血管炎)
• (b) 主要臨床所見3項目を満たすが、臨床経過の特徴を示さない場合(Churg-Strauss症候群)

• 5.参考となる検査所見

• (1) 白血球増加(1万/uL)
• (2) 血小板数増加(40万/uL)
• (3) 血清IgE増加(600U/mL 以上)
• (4) MPO-ANCA陽性
• (5) リウマトイド因子陽性
• (6) 肺浸潤陰影

これらの検査所見はすべての例に認められるとは限らない）

治療

• ステロイド
• 免疫抑制薬

予後

• 結節性多発動脈炎より予後良好
• 脳血管障害、呼吸不全、腸管出血、感染

参考

• 1. CSS nurs

http://www.nurs.or.jp/~academy/igaku/s4/s43235.htm

国試

• 101A054

「CSS」

　　[★]

• アレルギー性肉芽腫性血管炎, allergic granulomatous angiitis, AGA、チャーグ・ストラウス症候群, Churg-Strauss症候群, Churg-Strauss syndrome

参考

• CSS色見本

スタイルシート

http://hogehoge.tk/webdev/color/

「AGA」

　　[★] アレルギー性肉芽腫性血管炎, allergic granulomatous angiitis

「granulomatous angiitis」

　　[★]

肉芽腫性血管炎

関

central nervous system angiitis、central nervous system vasculitis、cerebral angiitis、cerebral vasculitis、CNS vasculitis、granulomatous arteritis

「allergic」

　　[★]

• adj.

• アレルギーの、アレルギー性の、アレルギー型の

関

allergy、hypersensitivity

　 　 　

「granulomatous」

　　[★]

• adj.

• 肉芽腫性の、肉芽腫の

関

granuloma