WordNet

characterized by or caused by allergy; "an allergic reaction"
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
inflammation of a blood vessel

PrepTutorEJDIC

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)』》

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/10/01 10:29:47」(JST)

wiki en

Vasculitis (plural: vasculitides)^[1] is a group of disorders that destroy blood vessels by inflammation.^[2] Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis.^[3] 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[edit]

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 causes 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.^[4] 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:^[5]

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, Golfer's vasculitis.

Conditions[edit]

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.

Several of these vasculitides are associated with antineutrophil cytoplasmic antibodies.^[6] These are

granulomatosis with polyangiitis (Wegener's granulomatosis)
eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
microscopic polyangiitis

Symptoms[edit]

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

Possible symptoms include:^[7]

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[edit]

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[edit]

Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, corticosteroids 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.

References[edit]

^ "Vasculitis - Definition from the Merriam-Webster Online Dictionary". Retrieved 2009-01-08.
^ "Glossary of dermatopathological terms. DermNet NZ". Retrieved 2009-01-08.
^ "Vasculitis" at Dorland's Medical Dictionary
^ Jennette JC, Falk RJ, Andrassy K, et al. (1994). "Nomenclature of systemic vasculitides. Proposal of an international consensus conference". Arthritis Rheum. 37 (2): 187–92. doi:10.1002/art.1780370206. PMID 8129773.
^ http://www.merckmanuals.com/professional/musculoskeletal_and_connective_tissue_disorders/vasculitis/overview_of_vasculitis.html
^ Millet A, Pederzoli-Ribeil M, Guillevin L, Witko-Sarsat V, Mouthon L (2013) Antineutrophil cytoplasmic antibody-associated vasculitides: is it time to split up the group? Ann Rheum Dis
^ "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". Retrieved 2009-05-07.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 皮膚小血管性血管炎の概要 overview of cutaneous small vessel vasculitis
2. 薬疹 drug eruptions
3. 小児血管炎：分類および発生率 vasculitis in children classification and incidence
4. 小児における過敏性血管炎 hypersensitivity vasculitis in children
5. 好酸球性肉芽腫性多発血管炎（チャーグ・ストラウス症候群）の治療および予後 treatment and prognosis of eosinophilic granulomatosis with polyangiitis churg strauss

English Journal

Elevated serum HMGB1 levels in patients with Henoch-Schönlein purpura.

Chen T1, Guo ZP, Wang WJ, Qin S, Cao N, Li MM.Author information 1Department of Dermatovenereology, West China Hospital of Sichuan University, Chengdu, 610041, Sichuan, China; Department of Dermatovenereology, Chengdu second people's hospital, Chengdu, 610041, Sichuan, China.AbstractHigh mobility group box-1 (HMGB1), has been implicated as a pro-inflammatory cytokine in the pathogenesis of various inflammatory and autoimmune diseases. However, information about HMGB1 in Henoch-Schönlein purpura (HSP) is still unclear. Herein, we investigated the role of HMGB1 in patients with HSP and the pro-inflammatory effects of HMGB1 on human dermal microvascular endothelial cell line (HMEC-1). Serum HMGB1 levels in patients with HSP, together with patients with allergic vasculitis (AV) and urticarial vasculitis (UV) were detected by enzyme-linked immunosorbent assay (ELISA). HMEC-1 cells were treated with HMGB1 at concentrations ranging from 4 ng/ml to 100 ng/ml. Serum HMGB1 levels were significantly elevated in patients with HSP, AV and UV, when compared with those in control group. Moreover, abundant cytoplasmic expression of HMGB1 was observed in endothelial cells in lesional skin of HSP patients. By using membrane cytokine antibody array, we indicate that HMGB1 markedly induced TNF-α and IL-6 release in cultured supernatant. Furthermore, by real-time quantitative PCR and ELISA, the effects of HMGB1 on these cytokines production in HMEC-1 cells were established. Finally, western blot data revealed that HMGB1 can induce phosphorylation of inhibitor of κB-α (IκBα), and the nuclear translocation of nuclear factor-κB (NF-κB) p65 in HMEC-1 cells. In conclusion, this study provides first observations on the association of HMGB1 with HSP. We suggest that HMGB1 may be an important mediator of endothelial inflammation through the induction of TNF-α and IL-6 production and may play a crucial role in the pathogenesis of HSP. This article is protected by copyright. All rights reserved.
Experimental dermatology.Exp Dermatol.2014 Apr 23. doi: 10.1111/exd.12422. [Epub ahead of print]
High mobility group box-1 (HMGB1), has been implicated as a pro-inflammatory cytokine in the pathogenesis of various inflammatory and autoimmune diseases. However, information about HMGB1 in Henoch-Schönlein purpura (HSP) is still unclear. Herein, we investigated the role of HMGB1 in patients with
PMID 24758390

The diagnosis and classification of Henoch-Schönlein purpura: an updated review.

Yang YH1, Yu HH1, Chiang BL2.Author information 1Department of Pediatrics, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.2Department of Medical Research, National Taiwan University Hospital, Graduate Institute of Clinical Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address: gicmbor@ntu.edu.tw.AbstractHenoch-Schönlein purpura (HSP) is a common childhood systemic vasculitis with clinical characteristics of cutaneous palpable purpura, arthralgia/arthritis, bowel angina, and hematuria/proteinuria. HSP is identified mainly based on the above presentations. Combined with pathohistological findings of leukocytoclastic vasculitis (LCV) and IgA-immune deposits in vessel walls and/or glomeruli increase the diagnostic sensitivity and specificity. However, considering the accessibility of biopsy and some patients with atypical presentations, there are still medical unmet needs in HSP diagnosis. This article reviews the diagnosis of HSP including the aspects of classification criteria, differential diagnosis, and some laboratory findings as the biomarkers with diagnostic potential.
Autoimmunity reviews.Autoimmun Rev.2014 Apr-May;13(4-5):355-8. doi: 10.1016/j.autrev.2014.01.031. Epub 2014 Jan 12.
Henoch-Schönlein purpura (HSP) is a common childhood systemic vasculitis with clinical characteristics of cutaneous palpable purpura, arthralgia/arthritis, bowel angina, and hematuria/proteinuria. HSP is identified mainly based on the above presentations. Combined with pathohistological findings of
PMID 24424188

Cutaneous leukocytoclastic vasculitis associated with letrozole.

Digklia A1, Tzika E, Voutsadakis IA.Author information 1Centre Pluridisciplinaire d'Oncologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.AbstractAromatase inhibitors are increasingly used in the treatment of early and metastatic breast cancer. They can produce various skin adverse effects but are only rarely associated with cutaneous vasculitis. We report the first case of cutaneous vasculitis clearly associated with the use of aromatase inhibitor letrozole.
Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners.J Oncol Pharm Pract.2014 Apr;20(2):146-8. doi: 10.1177/1078155213480535. Epub 2013 May 14.
Aromatase inhibitors are increasingly used in the treatment of early and metastatic breast cancer. They can produce various skin adverse effects but are only rarely associated with cutaneous vasculitis. We report the first case of cutaneous vasculitis clearly associated with the use of aromatase inh
PMID 23676512

Japanese Journal

Combined Allergic Bronchopulmonary Aspergillosis and Eosinophilic Granulomatosis with Polyangiitis: Three Cases and a Review of the Literature

Internal Medicine 55(7), 793-797, 2016
NAID 130005141948

手術を施行したChurg-Strauss症候群に伴う好酸球性胆嚢炎の1例

日本臨床外科学会雑誌 75(9), 2537-2543, 2014
NAID 130005063165

大腸病変により確定診断し得たアレルギー性肉芽腫性血管炎の1例

Progress of Digestive Endoscopy 84(1), 152-153, 2014
NAID 130004985492

Related Pictures

★リンクテーブル★

リンク元	「IgA血管炎」「アレルギー性血管炎」
関連記事	「allergic」

「IgA血管炎」

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

　　[★]

英: IgA vasculitis IgAV
同: アナフィラクトイド紫斑病、ヘノッホ・シェーンライン紫斑病 Henoch-Schönlein purpura, Henoch-Schonlein purpura, HSP, Schonlein-Henoch purpura, Schonlein-Henoch purpura; ヘノッホ・シェンライン紫斑病、Henoch-Schonlein紫斑病、Henoch-Schonlein紫斑、シェンライン・ヘノッホ紫斑病、シェーンライン・ヘノッホ紫斑病、Schonlein-Henoch紫斑病; アナフィラキシー様紫斑病アナフィラクトイド紫斑病アナフィラクトイド紫斑、anaphylactoid purpura、アレルギー性紫斑病アレルギー性紫斑 allergic purpura purpura allergica、アレルギー性血管炎 allergic vasculitis、リウマチ性紫斑病 rheumatic purpura purpura rheumatica、ヘノッホ紫斑病
関: IgA腎症。血管性紫斑病、皮膚小血管性血管炎、血管炎、劇症紫斑病、血小板非減少性紫斑病

まとめ

先行する上気道感染後に出現する紫斑が特徴的な血管炎である。IgAによる免疫複合体の血管壁への沈着、およびこれに次いで起こるIII型アレルギーが本態と考えられている。症状はG²AP、すなわち消化管症状、糸球体腎炎、関節痛、紫斑である。血液検査では軽度の好中球増多・好酸球増多がみられ、血液中のIgAも上昇するが、血小板、補体、IgG,IgEは変化しない。

定義

CHCC2012

IgA1優位の免疫沈着を伴い、小血管(主に毛細血管、細静脈または細動脈)を障害する血管炎。しばしば、皮膚、消化管、そして高頻度に関節炎を来す。IgA腎症と区別できない糸球体腎炎を生ずる。

概念

全身性疾患
血管炎(血管性紫斑病の一つである)　←　皮膚小血管性血管炎の特殊型
二次的にIgAによる免疫複合体がメサンギウムに沈着し糸球体腎炎を引き起こす

病因

IgA免疫複合体が血管壁に沈着。III型アレルギーの機序により、補体や好中球による組織障害
1. 溶連菌感染

小児では上気道炎などが先行することが多い

2. 薬剤(ペニシリン、アスピリン)、食物(牛乳、卵)

疫学

秋冬に多い。3-7歳に好発。乳児、成人ではまれ。

病理

病変は真皮上層に限局
フィブリノイド変性を伴う白血球破砕性血管炎。
真皮上層の乳頭下血管に好中球の核破砕を伴う血管炎がみられる。←フィブリノイド壊死
蛍光抗体直接法では、血管壁周囲にIgA,C3の沈着を認める。

症状 (PED.805, NDE.137)

皮膚(purpuric rash)、消化管(abdominal pain)、関節(arthritis)、腎臓(glomerulonephritis)

HSPの症状は「GA²P」と覚えてみる？

紫斑

ほぼ全例
両側の下腿や足背を中心に、時には大腿～上肢～腹部にまで、直径数～10mm以内の浸潤をふれる点状出血・紫斑が播種状に生じる。ときに圧痛が見られる。(NDE.138)
丘疹状で触知可能(palpable purpura)

[show details]

写真

http://graphics8.nytimes.com/images/2007/08/01/health/adam/19832.jpg

http://www.umm.edu/graphics/images/en/19831.jpg

http://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura

血管神経性浮腫(Quinckeの浮腫)

1/3の症例
一過性の限局性浮腫。東部、顔面、手足、陰嚢

消化管

約半数～80%
腹部の疝痛様疼痛、悪心や嘔吐、吐血、下血

関節

約2/3の症例
足、膝、手、肘などの関節炎症状。関節の腫脹、疼痛

腎臓

半数の症例
紫斑病性腎炎

発症から一ヶ月以内人タンパク尿、血尿で始まる腎炎を合併。多くは顕微鏡的血尿。
急性腎炎～ネフローゼ
組織的にはメサンギウム増殖性腎炎を呈する。

診断

検査

血算

PED.805

血小板：正常～やや増加　　　←　　　血管炎が紫斑の原因であって、血小板減少が原因ではない。

凝固系