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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]
| Vasculitis | |
|---|---|
| Other names | Vasculitides[1] |
| Petechia and purpura on the lower limb due to infection-associated vasculitis. | |
| Pronunciation | |
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]
| 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] |
Causes
Risk factors
Triggers
Genetics
Virology
Mechanism
Diagnosis
Treatment
Outlook
Epidemiology
| 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 |
History
Society and culture
Special populations
Geriatrics
Pregnancy