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'''Vasculitis''' is  a general term for inflammation of blood vessel walls. Most vasculitides are autoimmune diseases and the treatment involves immunosuppression or immunomodulation.
{{TOC|right}}
'''Vasculitis''' is  a general term for inflammation of blood vessel walls, resulting in [[ischemia]] of distal tissues.<ref name=KF>{{citation
| contribution = Chapter 17: Vasculitis
| author = Katz P, Fauci AS
| title = Immunology of Rheumatic Diseases
| editor = Gupta S, Talal N
| publisher = Plenum | year = 1985}}, p. 465</ref> Most vasculitides are [[autoimmune disease]]s and the treatment involves immunosuppression or immunomodulation.


==Classification==
==Classification==
Vasculitides can be classified by the size of the blood vessel that they predominantly affect.<ref name="pmid8129773">{{cite journal |author=Jennette JC, Falk RJ, Andrassy K, ''et al'' |title=Nomenclature of systemic vasculitides. Proposal of an international consensus conference |journal=Arthritis Rheum. |volume=37 |issue=2 |pages=187-92 |year=1994 |pmid=8129773 |doi=}}</ref> The following is based on [http://content.nejm.org/cgi/content/full/337/21/1512/T1 Table 1] and [http://content.nejm.org/cgi/content/full/337/21/1512/T2 Table 2] of Jennette.<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>
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>


===Large vessel vasculitis===
{| class="wikitable"
The large vessel vasculitides are probably mediated by cellular immunity and generally have normal complement levels 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. |volume=349 |issue=2 |pages=160–9 |year=2003 |pmid=12853590 |doi=10.1056/NEJMra022694}}</ref> They are characterized histologically by giant cells.
|+ Classification of vasculitis
! colspan="2"| &nbsp;!! Examples!! &nbsp;
|-
| colspan="2"|<span style="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/>
Associated with Type IV, cellular (delayed), [[hypersensitivity]].<br/>
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"/>


'''[[Takayasu arteritis]]'''. Primarily affects the [[aorta]] and its main branches.
|| ''[[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]].


'''[[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.
''[[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.
|| &nbsp;
|-
| colspan="2"|<span style="font-size:larger">Medium vessel</span><ref name="pmid12853590"/><br/>
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>
* palpable purpura with necrosis
* livedo reticularis
* subcutaneous nodules
* ulceration
* digital ischemia
|| ''[[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.


===Medium vessel vasculitis===
''[[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.  
The medium vessel vasculitides have similar pathology to the large vessel vasculitides.<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>


'''[[Polyarteritis nodosa]]'''. Systemic necrotizing vasculitis and [[aneurysm]] formation affecting both medium and small arteries with sparing of the lungs and glomeruli. If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with Wegener's granulomatosis than to classic PAN.
''[[Thromboangiitis obliterans]]'' (Buerger's disease). Blood tests for inflammation (including the [[erythrocyte sedimentation rate]]) and autoimmunity are usually normal.


'''[[Kawasaki disease]]'''. Usually in children, it affects large, medium, and small vessels, prominently the coronary arteries. Associated with a mucocutaneous lymph node syndrome.  
''[[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.
|| &nbsp;
|-
| rowspan="2" valign="top"|<span style="font-size:larger">Small vessel</span><ref  name="pmid9366584"/><br/>
Associated with Type III, immediate [[hypersensitivity]].<br/>
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
| 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"/>
Dermatological findings include:<ref name="pmid21774714"/>
* purpura
* urticaria
|valign="top"|''ANCA''<br/>
[[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>


'''[[Isolated CNS 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.
With partial exception from microscopic polyangiitis, these vasculitides are associated with respiratory manifestations.<ref name="pmid9366584"/><ref name="pmid16876669"/>
|| ''[[Granulomatous 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.


===Small vessel vasculitis===
''[[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.
There are several vasculitides that affect small vessels.<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> Some small vessel vasculitides, "Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, and their localized forms (eg, pauci-immune necrotising and crescentic glomerulonephritis)," are associated with [[antineutrophil cytoplasmic antibody|antineutrophil cytoplasmic antibodies]].<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>


====Antineutrophil cytoplasmic antibodies associated====
''[[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"/>
Vasculitides associated with [[antineutrophil cytoplasmic antibody]], with partial exception from microscopic polyangiitis, 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
|| 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>
| 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> A [[systematic review]] of [[antineutrophil cytoplasmic antibody]] (ANCA) positive vasculitis identified best treatments depending on whether the goal is to induce remission or maintenance and depending 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>


'''[[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.
|-
| valign="top"|''Immune complex''<br/>
These vasculitides are associated with [[immune complex]]es. With partial exception from microscopic polyangiitis, are associated with dermatological manifestations.<ref name="pmid9366584"/>


'''[[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.
With the exception of [[Henoch-Schonlein purpura]], serum levels of [[complement system protein]]s may be low in these vasculitides.
||  ''[[Henoch-Schonlein purpura]]'' (HSP). Systemic vasculitis due to tissue deposition of IgA-containing immune complexes. This is the most common vasculitis in children.


'''[[Microscopic polyangiitis|Microscopic polyarteritis/polyangiitis]]'''. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes Wegener's 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
''Hypocomplementemic urticarial vasculitis''<ref name="pmid21774714"/>
| 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>


====Immune-complex associated====
''[[Essential cryoglobulinemic vasculitis]]''. Most often due to [[hepatitis C]] infection, immune complexes of cryoglobulins.
These vasculitides, 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 }} <!--Formatted by http://sumsearch.uthscsa.edu/cite/--></ref>


'''[[Henoch-Schonlein purpura]]'''. Systemic vasculitis due to tissue deposition of IgA-containing immune complexes. Biopsy of lesions shows inflammation of small vessels. It is considered a form of hypersensitivity vasculitis but is distinguished by prominent deposits of IgA. This is the most common vasculitis in children.
|| &nbsp;
|-
| colspan="2"|<span style="font-size:larger">Variable-vessel vasculitis (VVV)</span><br/>
...
|| ''[[Behcet's Syndrome|Behçet disease]]''.


'''[[Hypersensitivity vasculitis]]'''. Usually due to a hypersensitivity reaction to a known drug. There is presence of skin vaculitis with palpable petechiae or purpura. Biopsy of these lesions reveal inflammation of the small vessels, termed leukocytoclastic vasculitis, which is most prominent in postcapillary venules.
''[[Cogan syndrome]]''.
|| &nbsp;
|-
| colspan="2"|<span style="font-size:larger">Single-organ vasculitis (SOV)</span><br/>
...
||
''[[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.


'''[[Essential cryoglobulinemic vasculitis]]'''. Most often due to hepatitic C infection, immune complexes of cryoglobulins --- proteins that consists of immunoglobulins and complement and precipitate in the cold while dissolving upon rewarming --- are deposited in walls of capillaries, venules, or arterioles. Therefore, complement will be low with histology showing vessel inflammation with immune deposits.
''[[Cutaneous arteritis]]''


'''Vasculitis secondary to connective tissue disorders'''. Usually secondary to [[lupus erythematosus| systemic lupus erythematosus]] (SLE), [[rheumatoid arthritis]] (RA), relapsing [[polychondritis]], [[Behcet's Syndrome]], and other connective tissue disorders.
''[[Primary central nervous system vasculitis]]''


'''Vasculitis secondary to viral infection'''. Usually due to hepatitis B and C, HIV, cytomegalovirus, Epstein-Barr virus, and Parvo B19 virus.
''[[Isolated aortitis]]''
 
|| &nbsp;
|-
| colspan="2"|<span style="font-size:larger">Vasculitis associated with systemic disease</span><br/>
...
||
[[Connective tissue disease]]s such as [[lupus erythematosus| systemic lupus erythematosus]] (SLE), [[rheumatoid arthritis]] (RA), [[sarcoidosis]], and others.
 
|| &nbsp;
|-
| colspan="2"|<span style="font-size:larger">Vasculitis associated with probable etiology</span><br/>
...
||
''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]].
 
Syphilis-associated aortitis
 
Drug-associated immune complex vasculitis
 
Drug-associated ANCA-associated vasculitis
 
Cancer-associated vasculitis
|| &nbsp;
|-
 
|}


==Treatment==
==Treatment==
In general, treatment involves immunosuppression, usually starting with [[corticosteroid]]s but often using cytotoxic agents such as [[cyclophosphamide]] or [[methotrexate]] as well. [[Plasmapheresis]] may be useful. Immunologic therapies are evolving.  
In general, treatment involves immunosuppression, usually starting with [[corticosteroid]]s but often using cytotoxic agents such as [[cyclophosphamide]] or [[methotrexate]] as well. [[Plasmapheresis]] may be useful. Immunologic therapies are evolving.  
==References==
==References==
<references/>
<small>
<references>
 
</references>
</small>
 
[[Category:Suggestion Bot Tag]]

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Vasculitis is a general term for inflammation of blood vessel walls, resulting in ischemia of distal tissues.[1] Most vasculitides are autoimmune diseases and the treatment involves immunosuppression or immunomodulation.

Classification

Vasculitides can be classified by the size of the blood vessel that they predominantly affect.[2][3]

Classification of vasculitis
  Examples  
Large vessel[4]

Associated with Type IV, cellular (delayed), hypersensitivity.
May have Langhans giant cells on biopsy, normal levels of complement system proteins, and no autoantibodies.[5][4]

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.

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.

 
Medium vessel[4]

Dermatological findings include:[6]

  • palpable purpura with necrosis
  • livedo reticularis
  • subcutaneous nodules
  • ulceration
  • digital ischemia
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.

Kawasaki disease. Affects vessels of all sizes especially the coronary arteries. Usually in children and is associated with a mucocutaneous lymph node syndrome.

Thromboangiitis obliterans (Buerger's disease). Blood tests for inflammation (including the erythrocyte sedimentation rate) and autoimmunity are usually normal.

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.

 
Small vessel[3]

Associated with Type III, immediate hypersensitivity.
Affect the "dermal venules[7], mucosal arterioles, glomerular capillaries, and pulmonary alveolar capillaries."[3] Dermatological findings include:[6]

  • purpura
  • urticaria
ANCA

Antineutrophil cytoplasmic antibodies (ANCA) are associated with some small vessel vasculitides including their localized forms such as pauci-immune necrotising and crescentic glomerulonephritis.[8]

With partial exception from microscopic polyangiitis, these vasculitides are associated with respiratory manifestations.[3][8]

Granulomatous 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 c-ANCA, but not vice versa.

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.

Microscopic polyarteritis/polyangiitis. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes granulomatous polyangiitis since both are associated with ANCA and similar extrapulmonary manifestations. Patients do not usually have symptomatic or histologic respiratory involvement.[8]

Treatment depends on whether the goal is to induce remission or maintenance and depends on severity of the vasculitis.[9]
Immune complex

These vasculitides are associated with immune complexes. With partial exception from microscopic polyangiitis, are associated with dermatological manifestations.[3]

With the exception of Henoch-Schonlein purpura, serum levels of complement system proteins may be low in these vasculitides.

Henoch-Schonlein purpura (HSP). Systemic vasculitis due to tissue deposition of IgA-containing immune complexes. This is the most common vasculitis in children.

Hypocomplementemic urticarial vasculitis[6]

Essential cryoglobulinemic vasculitis. Most often due to hepatitis C infection, immune complexes of cryoglobulins.

 
Variable-vessel vasculitis (VVV)

...

Behçet disease.

Cogan syndrome.

 
Single-organ vasculitis (SOV)

...

Cutaneous leukocytoclastic angiitisUsually due to a hypersensitivity reaction to a known drug.[10] There is presence of skin vaculitis termed leukocytoclastic vasculitis with palpable petechiae or purpura. Most prominent in postcapillary venules.

Cutaneous arteritis

Primary central nervous system vasculitis

Isolated aortitis

 
Vasculitis associated with systemic disease

...

Connective tissue diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), sarcoidosis, and others.

 
Vasculitis associated with probable etiology

...

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.

Syphilis-associated aortitis

Drug-associated immune complex vasculitis

Drug-associated ANCA-associated vasculitis

Cancer-associated vasculitis

 

Treatment

In general, treatment involves immunosuppression, usually starting with corticosteroids but often using cytotoxic agents such as cyclophosphamide or methotrexate as well. Plasmapheresis may be useful. Immunologic therapies are evolving.

References

  1. Katz P, Fauci AS (1985), Chapter 17: Vasculitis, in Gupta S, Talal N, Immunology of Rheumatic Diseases, Plenum, p. 465
  2. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al. (2013). "2012 revised international chapel hill consensus conference nomenclature of vasculitides.". Arthritis Rheum 65 (1): 1-11. DOI:10.1002/art.37715. PMID 23045170. Research Blogging.
  3. 3.0 3.1 3.2 3.3 3.4 Jennette JC, Falk RJ (1997). "Small-vessel vasculitis". N. Engl. J. Med. 337 (21): 1512-23. PMID 9366584[e]
  4. 4.0 4.1 4.2 Weyand CM, Goronzy JJ (2003). "Medium- and large-vessel vasculitis". N. Engl. J. Med. 349 (2): 160–9. DOI:10.1056/NEJMra022694. PMID 12853590. Research Blogging.
  5. Rabb H, Colvin RB (2007). "Case records of the Massachusetts General Hospital. Case 31-2007. A 41-year-old man with abdominal pain and elevated serum creatinine". N. Engl. J. Med. 357 (15): 1531–41. DOI:10.1056/NEJMcpc079024. PMID 17928602. Research Blogging.
  6. 6.0 6.1 6.2 Kroshinsky D, Stone JH, Nazarian RM (2011). "Case records of the Massachusetts General Hospital. Case 22-2011. A 79-year-old man with a rash, arthritis, and ocular erythema.". N Engl J Med 365 (3): 252-62. DOI:10.1056/NEJMcpc1100929. PMID 21774714. Research Blogging.
  7. Chen KR, Carlson JA (2008). "Clinical approach to cutaneous vasculitis.". Am J Clin Dermatol 9 (2): 71-92. PMID 18284262.
  8. 8.0 8.1 8.2 Bosch X, Guilabert A, Font J (2006). "Antineutrophil cytoplasmic antibodies". Lancet 368 (9533): 404–18. DOI:10.1016/S0140-6736(06)69114-9. PMID 16876669. Research Blogging.
  9. Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA 298 (6): 655-69. DOI:10.1001/jama.298.6.655. PMID 17684188. Research Blogging.
  10. ten Holder SM, Joy MS, Falk RJ (2002). "Cutaneous and systemic manifestations of drug-induced vasculitis.". Ann Pharmacother 36 (1): 130-47. PMID 11816242.