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| ==Classification== | | ==Classification== |
| Vasculitides can be classified by the size of the blood vessel that they predominantly affect.<ref name="pmid23045170">{{cite journal| author=Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al.| title=2012 revised international chapel hill consensus conference nomenclature of vasculitides. | journal=Arthritis Rheum | year= 2013 | volume= 65 | issue= 1 | pages= 1-11 | pmid=23045170 | doi=10.1002/art.37715 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23045170 }} </ref><ref name="pmid9366584">{{cite journal |author=Jennette JC, Falk RJ |title=Small-vessel vasculitis |journal=N. Engl. J. Med. |volume=337 |issue=21 |pages=1512-23 |year=1997 |pmid=9366584 |doi=|url=http://content.nejm.org/cgi/content/full/337/21/1512}}</ref>
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| {| class="wikitable"
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| |+ Classification of vasculitis
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| ! colspan="2"| !! Examples!!
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| | colspan="2"|<span style="font-weight:bold;font-size:larger">Large vessel</span><ref name="pmid12853590">{{cite journal |author=Weyand CM, Goronzy JJ |title=Medium- and large-vessel vasculitis |journal=N. Engl. J. Med. |volume=349 |issue=2 |pages=160–9 |year=2003 |pmid=12853590 |doi=10.1056/NEJMra022694}}</ref><br/>
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| Associated with Type IV, cellular (delayed), [[hypersensitivity]].<br/>
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| May have [[Langhans giant cell]]s on biopsy, normal levels of [[complement system protein]]s, and no autoantibodies.<ref name="pmid17928602">{{cite journal |author=Rabb H, Colvin RB |title=Case records of the Massachusetts General Hospital. Case 31-2007. A 41-year-old man with abdominal pain and elevated serum creatinine |journal=N. Engl. J. Med. |volume=357 |issue=15 |pages=1531–41 |year=2007 |pmid=17928602 |doi=10.1056/NEJMcpc079024}}</ref><ref name="pmid12853590">{{cite journal| author=Weyand CM, Goronzy JJ| title=Medium- and large-vessel vasculitis. | journal=N Engl J Med | year= 2003 | volume= 349 | issue= 2 | pages= 160-9 | pmid=12853590
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| | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&email=badgett@uthscdsa.edu&retmode=ref&cmd=prlinks&id=12853590 | doi=10.1056/NEJMra022694 }}</ref>
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| || '''[[Takayasu arteritis]]'''. Primarily affects the [[aorta]] and its main branches. Patients are usually less than 50 years old. May be associated with [[anti-endothelial cell antibody]].
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| '''[[temporal arteritis|Giant cell (temporal) arteritis]]'''. Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. Patients are usually over 50 years old.
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| | colspan="2"|<span style="font-weight:bold;font-size:larger">Medium vessel</span><ref name="pmid12853590">{{cite journal |author=Weyand CM, Goronzy JJ |title=Medium- and large-vessel vasculitis |journal=N. Engl. J. Med. |volume=349 |issue=2 |pages=160–9 |year=2003 |pmid=12853590 |doi=10.1056/NEJMra022694}}</ref><br/>
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| Dermatological findings include:<ref name="pmid21774714">{{cite journal| author=Kroshinsky D, Stone JH, Nazarian RM| title=Case records of the Massachusetts General Hospital. Case 22-2011. A 79-year-old man with a rash, arthritis, and ocular erythema. | journal=N Engl J Med | year= 2011 | volume= 365 | issue= 3 | pages= 252-62 | pmid=21774714 | doi=10.1056/NEJMcpc1100929 | pmc= | url= }} </ref>
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| * palpable purpura with necrosis
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| * livedo reticularis
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| * subcutaneous nodules
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| * ulceration
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| * digital ischemia
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| || '''[[Polyarteritis nodosa]]'''. Systemic necrotizing vasculitis and [[aneurysm]] formation with sparing of the lungs and glomeruli. Blood tests for autoimmunity are usually normal; however, tests for inflammation (such as the [[erythrocyte sedimentation rate]]) may be abnormal.
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| '''[[Kawasaki disease]]'''. Affects vessels of all sizes especially the [[coronary artery|coronary arteries]]. Usually in children and is associated with a mucocutaneous lymph node syndrome.
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| '''[[Thromboangiitis obliterans]]''' (Buerger's disease). Blood tests for inflammation (including the [[erythrocyte sedimentation rate]]) and autoimmunity are usually normal.
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| '''[[Isolated central nervous system vasculitis]]'''. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy.
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| | rowspan="2" valign="top"|<span style="font-weight:bold;font-size:larger">Small vessel</span><ref name="pmid9366584">{{cite journal |author=Jennette JC, Falk RJ |title=Small-vessel vasculitis |journal=N. Engl. J. Med. |volume=337 |issue=21 |pages=1512-23 |year=1997 |pmid=9366584 |doi=|url=http://www.nejm.org/doi/full/10.1056/NEJM199711203372106}}</ref><br/>
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| Associated with Type III, immediate [[hypersensitivity]].<br/>
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| Affect the "dermal venules<ref name="pmid18284262">{{cite journal| author=Chen KR, Carlson JA| title=Clinical approach to cutaneous vasculitis. | journal=Am J Clin Dermatol | year= 2008 | volume= 9 | issue= 2 | pages= 71-92 | pmid=18284262
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| | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&retmode=ref&cmd=prlinks&id=18284262 }} </ref>, mucosal arterioles, glomerular capillaries, and pulmonary alveolar capillaries."<ref name="pmid9366584">{{cite journal |author=Jennette JC, Falk RJ |title=Small-vessel vasculitis |journal=N. Engl. J. Med. |volume=337 |issue=21 |pages=1512-23 |year=1997 |pmid=9366584 |doi=|url=http://content.nejm.org/cgi/content/full/337/21/1512}}</ref>
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| Dermatological findings include:<ref name="pmid21774714">{{cite journal| author=Kroshinsky D, Stone JH, Nazarian RM| title=Case records of the Massachusetts General Hospital. Case 22-2011. A 79-year-old man with a rash, arthritis, and ocular erythema. | journal=N Engl J Med | year= 2011 | volume= 365 | issue= 3 | pages= 252-62 | pmid=21774714 | doi=10.1056/NEJMcpc1100929 | pmc= | url= }} </ref>
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| * purpura
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| * urticaria
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| |valign="top"|'''ANCA'''<br/>
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| [[Antineutrophil cytoplasmic antibody|Antineutrophil cytoplasmic antibodies]] (ANCA) are associated with some small vessel vasculitides including their localized forms such as pauci-immune necrotising and crescentic glomerulonephritis.<ref name="pmid16876669">{{cite journal |author=Bosch X, Guilabert A, Font J |title=Antineutrophil cytoplasmic antibodies |journal=Lancet |volume=368 |issue=9533 |pages=404–18 |year=2006 |pmid=16876669 |doi=10.1016/S0140-6736(06)69114-9}}</ref>
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| With partial exception from microscopic polyangiitis, these vasculitides are associated with respiratory manifestations.<ref name="pmid9366584"/><ref name="pmid16876669">{{cite journal| author=Bosch X, Guilabert A, Font J| title=Antineutrophil cytoplasmic antibodies. | journal=Lancet | year= 2006 | volume= 368 | issue= 9533 | pages= 404-18 | pmid=16876669
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| | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=16876669 | doi=10.1016/S0140-6736(06)69114-9 }} <!--Formatted by http://sumsearch.uthscsa.edu/cite/--></ref>
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| || '''[[Granulomatous with polyangiitis]] (Wegener's granulomatosis)'''. Systemic vasculitis of medium and small arteries, including venules and arterioles. Produces granulomatous inflammation of the respiratory tracts and necrotizing, pauci-immune glomerulonephritis. Most common cause of saddle nose deformity in USA (nose flattened due to destruction of nasal septum by granulomatous inflammation). Almost all patients with Wegener's have [[antineutrophil cytoplasmic antibody|c-ANCA]], but not vice versa.
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| '''[[Churg-Strauss syndrome|Churg-Strauss arteritis]]'''. Affects medium and small vessels with vascular and extravascular granulomatosis. Classically involves arteries of lungs and skin, but may be generalized. The triad [[asthma]], [[eczema]] and renal abnormalities (e.g., red blood cell casts in urine) should raise suspicion, calling for an [[eosinophil]] count. Eosinophilia, with this clinical presentation, is grounds for a preliminary diagnosis, but immunologic confirmation is needed.
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| '''[[Microscopic polyangiitis|Microscopic polyarteritis/polyangiitis]]'''. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes granulomatous polyangiitis since both are associated with [[antineutrophil cytoplasmic antibody|ANCA]] and similar extrapulmonary manifestations. Patients do not usually have symptomatic or histologic respiratory involvement.<ref name="pmid16876669">{{cite journal| author=Bosch X, Guilabert A, Font J| title=Antineutrophil cytoplasmic antibodies. | journal=Lancet | year= 2006 | volume= 368 | issue= 9533 | pages= 404-18 | pmid=16876669
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| | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=16876669 | doi=10.1016/S0140-6736(06)69114-9 }}</ref>
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| || Treatment depends on whether the goal is to induce remission or maintenance and depends on severity of the vasculitis.<ref name="pmid17684188">{{cite journal |author=Bosch X, Guilabert A, Espinosa G, Mirapeix E |title=Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review |journal=JAMA |volume=298 |issue=6 |pages=655-69 |year=2007 |pmid=17684188 |doi=10.1001/jama.298.6.655}}</ref>
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| | valign="top"|'''Immune complex'''<br/>
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| These vasculitides are associated with [[immune complex]]es. With partial exception from microscopic polyangiitis, are associated with dermatological manifestations.<ref name="pmid9366584">{{cite journal| author=Jennette JC, Falk RJ| title=Small-vessel vasculitis. | journal=N Engl J Med | year= 1997 | volume= 337 | issue= 21 | pages= 1512-23 | pmid=9366584 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&email=badgett@uthscdsa.edu&retmode=ref&cmd=prlinks&id=9366584 }}</ref>
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| With the exception of [[Henoch-Schonlein purpura]], serum levels of [[complement system protein]]s may be low in these vasculitides.
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| || '''[[Henoch-Schonlein purpura]]''' (HSP). Systemic vasculitis due to tissue deposition of IgA-containing immune complexes. This is the most common vasculitis in children.
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| '''Hypocomplementemic urticarial vasculitis'''<ref name="pmid21774714">{{cite journal| author=Kroshinsky D, Stone JH, Nazarian RM| title=Case records of the Massachusetts General Hospital. Case 22-2011. A 79-year-old man with a rash, arthritis, and ocular erythema. | journal=N Engl J Med | year= 2011 | volume= 365 | issue= 3 | pages= 252-62 | pmid=21774714 | doi=10.1056/NEJMcpc1100929 | pmc= | url= }} </ref>
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| '''[[Essential cryoglobulinemic vasculitis]]'''. Most often due to [[hepatitis C]] infection, immune complexes of cryoglobulins.
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| | colspan="2"|<span style="font-weight:bold;font-size:larger">Variable-vessel vasculitis (VVV)</span><br/>
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| ...
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| || '''[[Behcet's Syndrome|Behçet disease]]'''.
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| '''[[Cogan syndrome]]'''.
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| | colspan="2"|<span style="font-weight:bold;font-size:larger">Single-organ vasculitis (SOV)</span><br/>
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| ...
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| '''[[Cutaneous leukocytoclastic angiitis]]'''Usually due to a hypersensitivity reaction to a known drug.<ref name="pmid11816242">{{cite journal| author=ten Holder SM, Joy MS, Falk RJ| title=Cutaneous and systemic manifestations of drug-induced vasculitis. | journal=Ann Pharmacother | year= 2002 | volume= 36 | issue= 1 | pages= 130-47 | pmid=11816242 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&retmode=ref&cmd=prlinks&id=11816242 }} </ref> There is presence of skin vaculitis termed [[leukocytoclastic vasculitis]] with palpable petechiae or purpura. Most prominent in postcapillary venules.
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| '''[[Cutaneous arteritis]]'''
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| '''[[Primary central nervous system vasculitis]]'''
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| '''[[Isolated aortitis]]'''
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| | colspan="2"|<span style="font-weight:bold;font-size:larger">Vasculitis associated with systemic disease</span><br/>
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| ...
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| [[Connective tissue disease]]s such as [[lupus erythematosus| systemic lupus erythematosus]] (SLE), [[rheumatoid arthritis]] (RA), [[sarcoidosis]], and others.
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| | colspan="2"|<span style="font-weight:bold;font-size:larger">Vasculitis associated with probable etiology</span><br/>
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| ...
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| '''Vasculitis secondary to viral infection'''. Usually due to [[hepatitis B virus]] and [[hepatitis C virus]], [[Human Immunodeficiency Virus Type 1]] (HIV), [[cytomegalovirus]], [[Epstein Barr virus]], and [[Parvovirus B19]].
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| Syphilis-associated aortitis
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| Drug-associated immune complex vasculitis
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| Drug-associated ANCA-associated vasculitis
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| Cancer-associated vasculitis
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| ==References== | | ==References== |
| {{Reflist|2}} | | {{Reflist|2}} |