Systemic vasculitis
Inflammation and necrosis of blood vessels, classified by vessel size.
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The systemic vasculitides are characterized by inflammation of blood vessel walls, leading to tissue necrosis.[1] Vasculitis may be limited to skin or other organs, or may be a multisystem disorder with multiple manifestations.[1] Inflammation of both veins and arteries that leads to tissue death and vessel narrowing is what the term necrotizing vasculitis, also called systemic necrotizing vasculitis, describes.[2]
Quick Facts
- Field
- Immunology, rheumatology
Facts from the source article.
Key facts and history
The American College of Rheumatology has classification criteria for seven primary vasculitides: polyarteritis nodosa, Churg-Strauss syndrome, Wegener granulomatosis, hypersensitivity vasculitis, Henoch-Schönlein purpura, giant cell arteritis, and Takayasu arteritis.[1] Acute arterial lesions that were invariably found in malignant nephrosclerosis were characterized by Karl Theodor Fahr in 1919.[3] Recognized triggers for these conditions include tumors, medications, allergic reactions, and infectious organisms.[4] The primary blood vessel diameter they affect determines whether systemic vasculitides are classified as small, medium, large, or variable.[5]
According to the 2012 Chapel Hill Consensus Conference, large vessel vasculitis can involve any size artery but more often than other vasculitides affects the aorta and its major branches.[6] Vessels of all sizes and any type can be affected by variable vessel vasculitis, with no single vessel type being predominantly involved.[6]
What the cited sources add
Sharma notes that the American College of Rheumatology criteria were designed for research studies, but are often used for diagnosis.[1] Sharma states that the nomenclature includes microscopic polyangiitis and defines it as vasculitis involving microscopic vessels.[1] Sharma reports that patients with active vasculitis often have leukocytosis, anemia, and thrombocytopenia.[1] Sharma explains that a definitive diagnosis of systemic vasculitis should be made by the presence of characteristic symptoms and signs of vasculitis and at least one of the following: histologic evidence of vasculitis, positive serology for ANCA, or specific indirect evidence of vasculitis.[1]
Sharma describes that patients presenting with severe life-threatening vasculitis should be treated with cyclophosphamide and steroids, with adjuvant plasma exchange.[1]
References
- Sharma, "Systemic Vasculitis", American Family Physician, March 1, 2011, p. 556–565. facts only, no text reused. aafp.org
- Wikipedia, "Systemic vasculitis" (CC BY-SA 4.0), which cites: "Prognosis and outcome of 26 patients with systemic necrotizing vasculitis admitted to the intensive care unit", Rheumatology, October 2003, p. 1183–8; Vashi, "Acute Care Handbook for Physical Therapists", Elsevier, 2014, p. 161–199 (we also read that source) en.wikipedia.org
- Wikipedia, "Systemic vasculitis" (CC BY-SA 4.0), which cites: R. Mellon, "Experimental Necrotizing Arteriolitis Induced by a Protein Cleavage Product.", Experimental Biology and Medicine, 1935, p. 92–95 (original not read by us) en.wikipedia.org
- Wikipedia, "Systemic vasculitis" (CC BY-SA 4.0), which cites: Vashi, "Acute Care Handbook for Physical Therapists", Elsevier, 2014, p. 161–199 (we also read that source) en.wikipedia.org
- Wikipedia, "Systemic vasculitis" (CC BY-SA 4.0), which cites: Ralli, "Pathophysiology and therapy of systemic vasculitides", EXCLI Journal, 2020, p. 817–854 (original not read by us) en.wikipedia.org
- Wikipedia, "Systemic vasculitis" (CC BY-SA 4.0), which cites: Jennette, "2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides", Arthritis & Rheumatism, December 27, 2012, p. 1–11; Jennette, "Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides", Clinical and Experimental Nephrology, September 27, 2013, p. 603–606 (original not read by us) en.wikipedia.org
Each numbered sentence above was checked against the source it cites before publication. Sentences citing Wikipedia adapt text from the English Wikipedia article Systemic vasculitis (contributors), used under CC BY-SA 4.0; this entry is available under the same licence. Other sources were used for facts only; no text or images are copied from them.
More in Vasculitis
Sources
Compiled from Wikipedia and the sources listed below. Text from Wikipedia is available under CC BY-SA 4.0; this entry is adapted from it.
- Wikipedia: Systemic vasculitis (CC BY-SA 4.0).
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