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Medical condition From Wikipedia, the free encyclopedia

Vasculitis refers to the inflammation and destruction of blood vessels. Vasculitides are an uncommon and diverse group of disorders.[2]

Other namesVasculitides[1]
Pronunciation
Quick facts Vasculitis, Other names ...
Vasculitis
Other namesVasculitides[1]
Petechia and purpura on the lower limb due to infection-associated vasculitis.
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Signs and symptoms

The clinical presentation of the various vasculitides on the skin and internal organs is mostly determined by the diameter or size of the vessels mainly affected.[3] Non-specific symptoms are common and include fever, headache, fatigue, myalgia, weight loss, and arthralgia.[4][5]

All forms of vasculitis, even large vessel vasculitides, may cause skin manifestations. The most common skin manifestations include purpura, nodules, livedo reticularis, skin ulcers, and purpuric urticaria.[6]

More information Type, Name ...
Type Name Main symptoms
Primary large vessel vasculitis[7] Takayasu arteritis Diminished or absent pulses, vascular bruits, hypertension, Takayasu retinopathy, and aortic regurgitation.[8]
Giant cell arteritis Headache, scalp tenderness, jaw claudication, and blindness.[9]
Primary medium vessel vasculitis[7] Polyarteritis nodosa Mononeuritis multiplex, nodules, purpura, livedo, and hypertension.[10]
Kawasaki disease Fever, conjunctivitis, exanthema, palmoplantar erythema, cervical lymphadenopathy, and mucosal enanthema.[11][12]
Primary small vessel antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis[7] Microscopic polyangiitis Focal segmental rapidly progressive glomerulonephritis, proteinuria, hemoptysis, palpable purpura, abdominal pain, and peripheral neuropathy.[13]
Granulomatosis with polyangiitis Crusting rhinorrhea, sinusitis, chronic otitis

media, nasal obstruction, shortness of breath, and chronic cough.[14][15]

Eosinophilic granulomatosis with polyangiitis Asthma, allergic rhinitis, sinusitis, nasal polyps, peripheral neuropathy, pulmonary infiltrates, and abdominal pain.[16][17]
Primary immune complex small vessel vasculitis[7] Anti-glomerular basement membrane disease Glomerulonephritis, lung hemorrhage, hematuria, hemoptysis, cough, and dyspnea.[18]
Cryoglobulinemic vasculitis Palpable purpura, Raynaud's phenomenon, joint pain,[19] and peripheral neuropathy.
IgA vasculitis Palpable purpura, arthralgia, abdominal pain, nephritis, and haematuria.[20]
Hypocomplementemic urticarial vasculitis Hives, arthralgia, membranoproliferative glomerulonephritis, and chronic obstructive pulmonary disease.[21]
Primary variable vessel vasculitis[7] Behcet’s disease Oral ulcers, genital ulcers, papulopustular lesions, uveitis, superficial venous thrombosis and deep vein thrombosis.[22]
Cogan’s syndrome Interstitial keratitis, ocular redness, vertigo, and tinnitus.[23]
Single-organ vasculitis[24][7] Cutaneous small-vessel vasculitis Palpable purpura, necrosis, ulceration, bullae, and nodules.[25]
Cutaneous arteritis Nodules, livedo reticularis, ulcers, and gangrene.[26]
Primary central nervous system vasculitis Headache, cognitive impairment, stroke, encephalopathy, and seizures.[27]
Retinal vasculitis Visual impairments, floaters, and macular edema.[28]
Secondary vasculitis[7] Lupus vasculitis Palpable purpura, petechiae, papulonodular lesions, urticaria lesions, and mononeuritis multiplex.[29]
Rheumatoid vasculitis Purpura, focal digital lesions, ulcers, digital necrosis, pyoderma, distal sensory or motor neuropathy, and mononeuritis multiplex.[30]
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Causes


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Mechanism


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Outlook


Epidemiology

More information Type, Age ...
Type Age Sex Ethnic differences Incidence Prevalence
Takayasu arteritis 25 ± 12.5[31] 88.3% female, 11.6 % male[31] More common in Southeast Asia[32] Annual 0.4 - 3.4 per million[32] 8.4 - 40 per million[32]
Giant cell arteritis 71-80 years[33] Female to male ratio of 3:1[33] More common in Caucasians.[34] Annual 15–35 per 100 000 individuals 50< years[34] 84 - 250 per 100,000[32]
Polyarteritis nodosa 40–60 years[32] male-to-female ratio of 1.5:1[32] None[32] Annual incidence of 0.9–8.0 per million[32] 31 per million[32]
Kawasaki disease <5 years[32] Male to female ratio of 1.5:1[35] More common in Japan[32] 5 - 22 per 100,000 children < 5 years old in North America, Europe, and Australia. > 50 per 100,000 children < 5 years old in Japan, South Korea and Taiwan.[36]
Microscopic polyangiitis 66.98 ±13.12[37] No difference.[37] More common in Southern Europe.[38] 2.4 per million (Turkey) - 16 per million (USA).[38] 19.3 per million (Turkey) - 184 per million (USA).[38]
Granulomatosis with polyangiitis 55–69 years old.[39] Incidence higher in men.[39] Incidence is higher amongst white people.[39] 11.8/million person-years.[39] 134.9 per million.[39]
Eosinophilic granulomatosis with polyangiitis 57.4±14.2[40] 1.2 per 1,000,000 person-years.[40] 2.0–38.0 per 1,000,000 people.[40]
Anti-glomerular basement membrane disease
Cryoglobulinemic vasculitis
IgA vasculitis <16 years[32]
Hypocomplementemic urticarial vasculitis 40–60 years[32]
Behcet’s disease More common during the second and third decades[32]
Cogan’s syndrome
Cutaneous small-vessel vasculitis
Cutaneous arteritis
Primary central nervous system vasculitis
Retinal vasculitis
Lupus vasculitis
Rheumatoid vasculitis
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