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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

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.^[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

Conditions

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

Comparison of major types of vasculitis^[6]
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

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:^[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

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.
The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, 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.

References

^ "Vasculitis - Definition from the Merriam-Webster Online Dictionary". http://www.merriam-webster.com/dictionary/Vasculitis. Retrieved 2009-01-08.
^ "Glossary of dermatopathological terms. DermNet NZ". http://dermnetnz.org/pathology/pathology-glossary.html. 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
^ Unless else specified in boxes, then reference is:
- Stevens & Lowe: Pathology. At Fleshandbones.com
^ "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". http://vasculitis.med.jhu.edu/whatis/symptoms.html. Retrieved 2009-05-07.

UpToDate Contents

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1. 成人の血管炎の分類とそれに対するアプローチ classification of and approach to the vasculitides in adults
2. 成人における血管炎のマネージメントの概要 overview of the management of vasculitis in adults
3. 小児血管炎：マネージメントの概要 vasculitis in children management overview
4. Overview of cutaneous small vessel vasculitis
5. 小児血管炎：分類および発生率 vasculitis in children classification and incidence

English Journal

Can Wear Explain the Histological Variation Around Metal-on-metal Total Hips?

Ebramzadeh E1, Campbell P, Tan TL, Nelson SD, Sangiorgio SN.
Clinical orthopaedics and related research.Clin Orthop Relat Res.2015 Feb;473(2):487-94. doi: 10.1007/s11999-014-3874-5.
BACKGROUND: There is a general perception that adverse local tissue reactions in metal-on-metal hip arthroplasties are caused by wear, but the degree to which this is the case remains controversial.QUESTIONS/PURPOSES: To what extent is the magnitude of wear associated with (1) the histological chang
PMID 25141844

Connective Tissue Disorders Associated with Vasculitis and Vaso-Occlusive Disease of the Hand.

Michelotti B1, Rizzo M2, Moran SL3.
Hand clinics.Hand Clin.2015 Feb;31(1):63-73. doi: 10.1016/j.hcl.2014.09.006. Epub 2014 Nov 25.
Hand ischemia caused by vasculitis is a secondary finding in many autoimmune processes. Many of these autoimmune diseases are managed primarily with medications that can prevent the development of occlusive disease, tissue ischemia, and tissue loss. Unfortunately several disease conditions can be re
PMID 25455357

Hydrophilic polymer embolism and associated vasculopathy of the lung: prevalence in a retrospective autopsy study.

Mehta RI1, Mehta RI2, Choi JM3, Mukherjee A4, Castellani RJ3.
Human pathology.Hum Pathol.2015 Feb;46(2):191-201. doi: 10.1016/j.humpath.2014.09.011. Epub 2014 Oct 7.
Hydrophilic polymers are commonly applied as surface coatings on vascular devices and have been shown to dissociate during endovascular use, causing hydrophilic polymer embolism (HPE). Adverse effects related to this phenomenon have been recognized and reported. The prevalence of this complication i
PMID 25543660

Japanese Journal

感音難聴と顔面神経麻痺を示すMPO-ANCA関連中耳疾患の1例 : 診断基準案の提案

武田憲昭,神村盛一郎,千田いづみ,北村嘉章,陣内自治,阿部晃治,田村公一,宇高二良,遠藤亜紀
Otology Japan 21(5), 808-815, 2011-12-26
NAID 10030285311

ANCA関連血管炎と肺病変 (特集自己免疫疾患と肺疾患)

山田秀裕,前田聡彦,山崎宣興
呼吸器内科 20(5), 396-401, 2011-11
NAID 40019140228

Related Pictures

Systemic Vasculitis Syndromes Part 1 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides Vasculitis | The BMJ Vasculitis and Thrombophlebitis: Background, Classification Guidelines, The EULAR and Vasculitis y riñon ROBBINS, VESSELS, TEST 2 - StudyBlue

★リンクテーブル★

リンク元

「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: angiitis
関: 脈管炎 vasculitis　vasculitides、血管内膜炎、血管炎症候群

分類

CHCC2012

large vessel vasculitis LVV 大型血管炎	Takayasu arteritis TAK 高安動脈炎
	giant cell arteritis GCA 巨細胞性動脈炎
medium vessel vasculitis MVV 中型血管炎	polyarteritis nodosa PAN 結節性多発動脈炎
	Kawasaki disease KD 川崎病
small vessel vasculitis SVV 小型血管炎	antineutrophil cytoplasmic antibody-associated vasculitis AAV 抗好中球細胞質抗体関連血管炎	microscopic polyangiitis MPA 顕微鏡的多発血管炎
		granulomatosis with polyangiitis GPA 多発血管炎性肉芽腫症(Wegener肉芽腫症)
		eosinophilic granulomatosis with polyangiitis EGPA 好酸球性多発血管炎性肉芽腫症(Churg-Strauss症候群)
	immune complex SVV 免疫複合体性小型血管炎	anti-glomerular basement membrane disease 抗糸球体基底膜抗体病(抗GBM病)
		cryoglobulinemic vasculitis CV クリオグロブリン血症性血管炎
		IgA vasculitis IgAV IgA血管炎(Henoch-Schonlein紫斑病)
		hypocomplementemic urticarial vasculitis HUV 低補体血症性蕁麻疹様血管炎(抗C1q血管炎)
variable vessel vasculitis VVV 多様な血管を侵す血管炎	Behcet's disease BD Behcet病
	Cogan's syndrome CS Cogan症候群
single-organ vasculitis SOV 単一臓器血管炎	cutaneous leukocytoclastic angiitis 皮膚白血球破砕性血管炎
	cutaneous arteritis 皮膚動脈炎
	primary central nervous system vasculitis 原発性中枢神経系血管炎
	isolated aortitis 限局性大動脈炎
vasculitis associated with systemic disease 全身性疾患関連血管炎	lupus vasculitis ループス血管炎
	rheumatoid vasculitis リウマトイド血管炎
	sarcoid vasculitis サルコイド血管炎
vasculitis associated with probable etiology 推定病因を有する血管炎	hepatitis C virus-associated cryoglobulinemic vasculitis C型肝炎ウイルス関連クリオグロブリン血症性血管炎
	hepatitis B virus-associated vasculitis B型肝炎ウイルス関連血管炎
	syphilis-associated aortitis 梅毒関連大動脈炎
	drug-associated immune complex vasculitis 薬剤関連免疫複合体性血管炎
	drug-associated ANCA-associated vasculitis 薬剤関連ANCA関連血管炎
	cancer-associated vasculitis がん関連血管炎

参考

血管炎症候群 - 日本内科学会雑誌第103巻第10号

1. 血管炎症候群の診療ガイドライン

http://www.j-circ.or.jp/guideline/pdf/JCS2008_ozaki_h.pdf

血管炎・血管障害診療ガイドライン 2016 年改訂版 - 日本皮膚科学会

https://www.dermatol.or.jp/uploads/uploads/files/vasculitisGL.pdf

[urlVasculitis_-_Definition_from_the_Merriam-Webster_Online_Dictionary-1] "Vasculitis - Definition from the Merriam-Webster Online Dictionary". http://www.merriam-webster.com/dictionary/Vasculitis. Retrieved 2009-01-08.

[urlGlossary_of_dermatopathological_terms._DermNet_NZ-2] "Glossary of dermatopathological terms. DermNet NZ". http://dermnetnz.org/pathology/pathology-glossary.html. Retrieved 2009-01-08.

[3] "Vasculitis" at Dorland's Medical Dictionary

[pmid8129773-4] 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.

[5] ttp://www.merckmanuals.com/professional/musculoskeletal_and_connective_tissue_disorders/vasculitis/overview_of_vasculitis.html

[6] Unless else specified in boxes, then reference is:

Stevens & Lowe: Pathology. At Fleshandbones.com

[7] Stevens & Lowe: Pathology. At Fleshandbones.com

[urlThe_Johns_Hopkins_Vasculitis_Center_-_Symptoms_of_Vasculitis-7] "The Johns Hopkins Vasculitis Center - Symptoms of Vasculitis". http://vasculitis.med.jhu.edu/whatis/symptoms.html. Retrieved 2009-05-07.

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vasculitides