Vasculitis
Basics
Description
Description
- Injury to the walls of blood vessels from inflammation:
- Ischemia and necrosis
- Aneurysms and hemorrhage
- Immunopathologic mechanisms:
- Deposition of circulating antigen–antibody complex and complement fixation
- Cell-mediated hypersensitivity
- Granulomatous tissue reaction from persistent inflammation and formation of epithelioid giant cells
- Vasculitides represent a wide group of disorders:
- Multisystem disease with constitutional symptoms and inflammatory lab indices
- Secondary to another disorder or trigger, or primary if vasculitis is the principal feature and the cause is unknown
- Multiple factors determine presentation:
- The size of the affected blood vessels
- The specific distribution, severity, and duration of the inflammation
- 1 out of 2,000 adults has some form of vasculitis
Etiology
Etiology
- Largest arteries:
- Giant cell arteritis:
- Granulomatous arteritis of the aorta and its major branches often involving the temporal artery
- Patients >50 yr
- Takayasu arteritis:
- Granulomatous inflammation of the aorta and its major branches
- Usually occurs in patients <50 yr
- Giant cell arteritis:
- Medium-sized arteries:
- Polyarteritis nodosa (PAN):
- Small- and medium-sized arteritis
- Common distribution includes vessels supplying the muscles, joints, intestines, nerves, kidneys, and skin
- Most common in middle age
- Kawasaki disease (mucocutaneous lymph node syndrome):
- Coronary arteries are often involved and involves large-, medium-, and small-sized arteries
- Usually occurs in children
- Polyarteritis nodosa (PAN):
- Small- and medium-sized arteries:
- Granulomatosis with polyangiitis (Wegener granulomatosis):
- Necrotizing vasculitis affecting small- to medium-sized vessels
- Granulomatous inflammation of the upper and lower respiratory tract and glomerulonephritis
- Microscopic polyangiitis:
- Necrotizing affecting small vessels
- Glomerulonephritis is very common
- Pulmonary capillaritis often occurs
- Eosinophilic granulomatosis with polyangiitis (Churg–Strauss):
- Small- and medium-sized arteries
- Mainly lungs, GI, and nerves
- Can also involve heart, skin, and kidney
- Granulomatosis with polyangiitis (Wegener granulomatosis):
- Small arteries:
- IgA vasculitis (Henoch–Schönlein):
- Most patients <20 yr
- Buerger disease (thromboangiitis obliterans)
- Cryoglobulinemic vasculitis
- Anti-GBM disease (Goodpasture)
- Vasculitis of lupus, rheumatoid arthritis, Sjögren
- Drug-induced vasculitis:
- IgA vasculitis (Henoch–Schönlein):
- Arteries and veins of various size:
- Behçet disease
- Relapsing polychondritis
- Secondary vasculitides:
- Bacterial infections:
- Streptococcal, tuberculous, staphylococcal, Lyme disease, leprosy
- Viral infections:
- Hepatitis B or C, CMV, HSV, HIV
- Rickettsial infections
- Hairy cell leukemia
- Lymphoma
- Serum sickness
- Bacterial infections:
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Citation
Schaider, Jeffrey J., et al., editors. "Vasculitis." 5-Minute Emergency Consult, 6th ed., Lippincott Williams & Wilkins, 2020. Emergency Central, emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307249/all/Vasculitis.
Vasculitis. In: Schaider JJJ, Barkin RMR, Hayden SRS, et al, eds. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307249/all/Vasculitis. Accessed December 3, 2024.
Vasculitis. (2020). In Schaider, J. J., Barkin, R. M., Hayden, S. R., Wolfe, R. E., Barkin, A. Z., Shayne, P., & Rosen, P. (Eds.), 5-Minute Emergency Consult (6th ed.). Lippincott Williams & Wilkins. https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307249/all/Vasculitis
Vasculitis [Internet]. In: Schaider JJJ, Barkin RMR, Hayden SRS, Wolfe RER, Barkin AZA, Shayne PP, Rosen PP, editors. 5-Minute Emergency Consult. Lippincott Williams & Wilkins; 2020. [cited 2024 December 03]. Available from: https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307249/all/Vasculitis.
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