Highlights & Basics
- The systemic vasculitides are subacute illnesses associated with signs and symptoms of inflammation that can affect virtually any organ system.
- Many of these diseases have typical patterns of organ involvement.
- The diagnostic value of a biopsy, performed early in the course of illness, cannot be overstated. However, biopsy may not always be possible due to the site of affected tissue.
- Treatment depends on the type of vasculitis and the severity of the clinical manifestations, but typically involves a corticosteroid and/or other immunosuppressive therapy.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Chest CT demonstrating cavitary pulmonary lesion secondary to granulomatosis with polyangiitis, a common form of antineutrophil cytoplasmic autoantibody-associated vasculitis
Biopsy specimen showing florid transmural inflammation of a small artery
Photomicrograph of the aorta from a patient with Takayasu arteritis demonstrating marked thickening of the intimal layer and inflammatory infiltrates in the media and laminar necrosis
A purpuric rash
Citations
Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16;2022-223764.[Abstract][Full Text]
Hellmich B, Agueda A, Monti S, et al. 2018 update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30.[Abstract][Full Text]
Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021 Aug;73(8):1366-83.[Abstract][Full Text]
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