Vasculitis
Vasculitis (plural: vasculitides)[1] refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels.[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
There are many ways to classify vasculitis.
- It can be classified by the 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.
- It can be classified by the 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").
- Vasculitides can be classified by the 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, it should be noted that there can be some variation in the size of the vessels affected.
Conditions
Some disorders have vasculitis as their main feature. The major types are given in the table below:
| Comparison of major types of vasculitis[5] |
| 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 are sometimes classified as vasculitis as well, but rather belong to arteritis since they mainly involve arteries.
Furthermore, there are many conditions that have vasculitis as an accompanying or atypical symptom, including:
Symptoms
Possible symptoms include: [6]
- 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
- 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.
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 cefalexin 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
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Diseases of the skin and appendages by morphology |
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| Growths |
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Pigmented
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Dermal and
subcutaneous
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epidermal inclusion cyst · hemangioma · dermatofibroma · keloid · lipoma · neurofibroma · xanthoma · Kaposi's sarcoma · infantile digital fibromatosis · granular cell tumor · leiomyoma · lymphangioma circumscriptum · myxoid cyst |
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| Rashes |
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With
epidermal
involvement
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Eczematous
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Papular
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scabies · insect bite reactions · lichen planus · miliaria · keratosis pilaris · lichen spinulosus · transient acantholytic dermatosis · lichen nitidus · pityriasis lichenoides et varioliformis acuta
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Hypopigmented
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tinea versicolor · vitiligo · pityriasis alba · postinflammatory hyperpigmentation · tuberous sclerosis · idiopathic guttate hypomelanosis · leprosy · hypopigmented mycosis fungoides |
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Without
epidermal
involvement
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Red
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Blanchable
Erythema
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Generalized
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Localized
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cellulitis · abscess · boil · erythema nodosum · carcinoid syndrome · fixed drug eruption
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Specialized
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urticaria · erythema (multiforme · migrans · gyratum repens · annulare centrifugum · ab igne)
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Nonblanchable
Purpura
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Macular
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thrombocytopenic purpura · actinic purpura
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Papular
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Indurated
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scleroderma/morphea · granuloma annulare · lichen sclerosis et atrophicus · necrobiosis lipoidica
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Miscellaneous
disorders |
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Ulcers
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telogen effluvium · androgenic alopecia · trichotillomania · alopecia areata · systemic lupus erythematosus · tinea capitis · loose anagen syndrome · lichen planopilaris · folliculitis decalvans · acne keloidalis nuchae |
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aphthous stomatitis · oral candidiasis · lichen planus · leukoplakia · pemphigus vulgaris · mucous membrane pemphigoid · cicatricial pemphigoid · herpesvirus · coxsackievirus · syphilis · systemic histoplasmosis · squamous cell carcinoma
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Cardiovascular disease: vascular disease · Circulatory system pathology (I70–I99, 440–456) |
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Arteries, arterioles
and capillaries |
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Arteritis (Aortitis) · Buerger's disease
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Arterial occlusive disease/
peripheral vascular disease
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Arteriosclerosis
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Atherosclerosis (Foam cell, Fatty streak, Atheroma, Intermittent claudication) · Monckeberg's arteriosclerosis · Arteriolosclerosis (Hyaline, Hyperplastic, oxycholesterol, cholesterol, LDL, trans fat)
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Stenosis
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Renal artery stenosis · Carotid artery stenosis
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Other
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torso: Aortic aneurysm (Thoracic aortic aneurysm, Abdominal aortic aneurysm) · Aortic dissection · Coronary artery aneurysm
head/neck: Cerebral aneurysm · Intracranial berry aneurysm · Carotid artery dissection · Vertebral artery dissection · Familial aortic dissection
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Vascular malformation
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Arteriovenous malformation · Arteriovenous fistula · Telangiectasia (Hereditary hemorrhagic telangiectasia)
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Spider angioma · Halo nevus · Cherry hemangioma
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| Veins |
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Phlebitis
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Venous thrombosis/
Thrombophlebitis
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primarily lower limb (Deep vein thrombosis)
abdomen (May-Thurner syndrome, Portal vein thrombosis, Budd–Chiari syndrome, Renal vein thrombosis)
upper limb/torso (Paget-Schroetter disease, Mondor's disease)
head (Cerebral venous sinus thrombosis)
Post-thrombotic syndrome
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Varicocele · Gastric varices · Portacaval anastomosis ( Hemorrhoid, Esophageal varices, Caput medusae)
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Other
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Superior vena cava syndrome · Inferior vena cava syndrome · Venous ulcer · Chronic venous insufficiency · Chronic cerebrospinal venous insufficiency
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| Arteries or veins |
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| Blood pressure |
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Hypertensive heart disease · Hypertensive nephropathy · Essential hypertension · Secondary hypertension (Renovascular hypertension) · Pulmonary hypertension · Malignant hypertension · Benign hypertension · Systolic hypertension · White coat hypertension |
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anat(a:h,u,t,a,l,v:h,u,t,a,l)/phys/devp/cell/
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proc, drug(C2s/,C3,C4,C5,,C8,C9)
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Cutaneous vasculitis (L95) |
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Cutaneous
vasculitis |
Livedoid vasculitis · Erythema elevatum diutinum · Capillaritis · Urticarial vasculitis · Nodular vasculitis
|
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Ungrouped
vascular-related
cutaneous
conditions |
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ICD C/D
|
purpura: Cryofibrinogenemic purpura · Cryoglobulinemic purpura · Drug-induced purpura · Drug-induced thrombocytopenic purpura · Food-induced purpura · Henoch–Schönlein purpura · Idiopathic thrombocytopenic purpura · Obstructive purpura · Orthostatic purpura · Purpura fulminans · Purpura secondary to clotting disorders · Purpuric agave dermatitis · Pigmentary purpuric eruptions · Solar purpura · Thrombotic thrombocytopenic purpura · Traumatic purpura · Waldenström hyperglobulinemic purpura
Paroxysmal nocturnal hemoglobinuria · Disseminated intravascular coagulation · Waldenström macroglobulinemia
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ICD I
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venous: Deep venous thrombosis · Venous insufficiency ulceration · Septic thrombophlebitis · Superficial thrombophlebitis · Mondor's disease
lymphatic: Bullous lymphedema · Factitial lymphedema · Lymphedema praecox · Lymphedema–distichiasis syndrome · Postinflammatory lymphedema · Postmastectomy lymphangiosarcoma · Secondary lymphedema · Hennekam syndrome · Aagenaes syndrome · Milroy's disease
telangiectasia: Generalized essential telangiectasia · Hereditary hemorrhagic telangiectasia · Unilateral nevoid telangiectasia
Raynaud phenomenon · Raynaud's disease · Thromboangiitis obliterans · Erythromelalgia · Postcardiotomy syndrome
|
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ICD M
|
systemic vasculitis: Giant-cell arteritis · Polyarteritis nodosa · Kawasaki's disease · Wegener granulomatosis · Microscopic polyangiitis · Takayasu arteritis
Bullous small vessel vasculitis · Cutaneous small-vessel vasculitis
Sneddon's syndrome
|
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Other/
ungrouped
|
ungrouped: Acroangiodermatitis · Acute hemorrhagic edema of infancy · Arterial insufficiency ulcer · Arteriosclerosis obliterans · Bier spots · Blueberry muffin baby · Bonnet–Dechaume–Blanc syndrome · Calciphylaxis · Cholesterol embolus · Cobb syndrome · Cryoglobulinemic vasculitis · Epidemic dropsy · Fibrinolysis syndrome · Hematopoietic ulcer · Livedo (Livedo reticularis · Livedoid dermatitis · Livedoid vasculopathy) · Malignant atrophic papulosis · Marshall–White syndrome · Neuropathic ulcer · Painful bruising syndrome · Parkes Weber syndrome · Paroxysmal hand hematoma · Perinatal gangrene of the buttock · Reactive angioendotheliomatosis · Sinusoidal hemangioma · Stasis dermatitis · Trousseau's syndrome · Yellow nail syndrome
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noco(i,,d,q,u,,p,,,v)/cong/tumr(n,e,d), sysi/
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Systemic CT disorders (M32-M36, 710) |
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| General |
Systemic lupus erythematosus: Drug-induced SLE · Libman-Sacks endocarditis
Inflammatory myopathy/Myositis: Dermatopolymyositis (Dermatomyositis/Juvenile dermatomyositis, Polymyositis) · Inclusion body myositis
Scleroderma: Systemic scleroderma (Progressive systemic sclerosis, CREST syndrome)
Overlap syndrome / Mixed connective tissue disease
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| Other hypersensitivity/autoimmune |
Sjögren's syndrome
|
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| Other |
Behçet's disease · Polymyalgia rheumatica · Eosinophilic fasciitis · Eosinophilia–myalgia syndrome · fibrillin ( Marfan syndrome, Congenital contractural arachnodactyly)
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anat (h/n, u, t/d, a/p, l)/phys/hist
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noco(m, s, c)/cong(d)/tumr, sysi/, injr
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Inflammation |
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| Acute |
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| Chronic |
Macrophage · Epithelioid cell · Giant cell · Granuloma
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| Processes |
Traditional: Rubor · Calor · Tumor · Dolor (pain) · Functio laesa
Modern: Acute-phase reaction/ Fever · Vasodilation · Increased vascular permeability · Exudate · Leukocyte extravasation · Chemotaxis |
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| Specific types |
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CNS ( Encephalitis, Myelitis) · Meningitis (Arachnoiditis) · PNS ( Neuritis) · eye (Dacryoadenitis, Scleritis, Keratitis, Choroiditis, Retinitis, Chorioretinitis, Blepharitis, Conjunctivitis, Iritis, Uveitis) · ear (Otitis, Labyrinthitis, Mastoiditis)
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mouth (Stomatitis, Gingivitis, Gingivostomatitis, Glossitis, Tonsillitis, Sialadenitis/Parotitis, Cheilitis, Pulpitis, Gnathitis) · tract (Esophagitis, Gastritis, Gastroenteritis, Enteritis, Colitis, Enterocolitis, Duodenitis, Ileitis, Caecitis, Appendicitis, Proctitis) · accessory ( Hepatitis, Cholangitis, Cholecystitis, Pancreatitis) · Peritonitis
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Dermatitis (Folliculitis) · Cellulitis · Hidradenitis |
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Arthritis · Dermatomyositis · soft tissue (Myositis, Synovitis/Tenosynovitis, Bursitis, Enthesitis, Fasciitis, Capsulitis, Epicondylitis, Tendinitis, Panniculitis)
Osteochondritis: Osteitis (Spondylitis, Periostitis) · Chondritis
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Nephritis (Glomerulonephritis, Pyelonephritis) · Ureteritis · Cystitis · Urethritis
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female: Oophoritis · Salpingitis · Endometritis · Parametritis · Cervicitis · Vaginitis · Vulvitis · Mastitis
male: Orchitis · Epididymitis · Prostatitis · Balanitis · Balanoposthitis
pregnancy/newborn: Chorioamnionitis · Omphalitis
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Insulitis · Hypophysitis · Thyroiditis · Parathyroiditis · Adrenalitis
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Lymphangitis · Lymphadenitis
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