Difference between revisions of "Vasculitides"

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Vasculitides (view source)

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=Vasculitides=
=Small vessel vasculitides=
The follow section has information specific to the individual types of vasculitis.
The follow section has information specific to the individual types of small vessel vasculitis.


==Small vessel leukocytoclastic vasculitis==
==Small vessel leukocytoclastic vasculitis==
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*PAS - look for fungus.
*PAS - look for fungus.


==Giant cell arteritis==
===General===
*[[AKA]] ''temporal arteritis''.


===Clinical===
Features:
*Classic finding: jaw claudication, in a patient older than 50 years.
*Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.
Work-up:
*CRP, ESR, temporal artery biopsy.
Treatment:
*Treat right away with high dose steroids.
**Biopsy is confirmatory.
===Microscopic===
Features:
*Classical: [[granulomas]].
Image(s):
*[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)].
==Takayasu arteritis==
===General===
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Disease of medium/large arteries.
**Classically involves the aortic arch (leading to decreased pulses in the upper limbs).
*Typically in patients <40 yrs old.
*Usually asian.
===Microscopic===
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
*Mononuclear inflammation in media.
*Granulomas, [[giant cells]].
*+/-Patchy necrosis of media.
==Polyarteritis nodosa==
*Abbreviated ''PAN''.
===General===
*Involves small and medium sized vessels.
*Often - renal vessels, mesenteric vessels.<ref name=Ref_Klatt14>{{Ref Klatt|14}}</ref>
*Strong association with ''hepatitis B'' (see [[medical liver diseases]]); ~1/3 of patients with PAN have HBV.
Serology:
*ANCA is usually negative.
===Microscopic===
Features - medium-sized vessels with:
*Inflammatory cells (neutrophils, lymphocytes) within the tunica media.
*Fibroid necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).
**Usu. focal (wall) involvement; classically leads to berry microaneurysms - ergo the name ''polyarteritis nodosa''.
Image:
*[http://www.immunologyclinic.com/figure.asp?chap=10&fig=14-05d PAN (immunologyclinic.com)].


==Microscopic polyangiitis==
==Microscopic polyangiitis==
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*Eosinophilic vasculitis associated with a CTD.<ref name=pmid8708015>{{Cite journal  | last1 = Chen | first1 = KR. | last2 = Su | first2 = WP. | last3 = Pittelkow | first3 = MR. | last4 = Conn | first4 = DL. | last5 = George | first5 = T. | last6 = Leiferman | first6 = KM. | title = Eosinophilic vasculitis in connective tissue disease. | journal = J Am Acad Dermatol | volume = 35 | issue = 2 Pt 1 | pages = 173-82 | month = Aug | year = 1996 | doi =  | PMID = 8708015 }}</ref>
*Eosinophilic vasculitis associated with a CTD.<ref name=pmid8708015>{{Cite journal  | last1 = Chen | first1 = KR. | last2 = Su | first2 = WP. | last3 = Pittelkow | first3 = MR. | last4 = Conn | first4 = DL. | last5 = George | first5 = T. | last6 = Leiferman | first6 = KM. | title = Eosinophilic vasculitis in connective tissue disease. | journal = J Am Acad Dermatol | volume = 35 | issue = 2 Pt 1 | pages = 173-82 | month = Aug | year = 1996 | doi =  | PMID = 8708015 }}</ref>


=Medium vessel vasculitides=
The follow section has information specific to the individual types of medium vessel vasculitis.
==Polyarteritis nodosa==
*Abbreviated ''PAN''.
===General===
*Involves small and medium sized vessels.
*Often - renal vessels, mesenteric vessels.<ref name=Ref_Klatt14>{{Ref Klatt|14}}</ref>
*Strong association with ''hepatitis B'' (see [[medical liver diseases]]); ~1/3 of patients with PAN have HBV.
Serology:
*ANCA is usually negative.
===Microscopic===
Features - medium-sized vessels with:
*Inflammatory cells (neutrophils, lymphocytes) within the tunica media.
*Fibroid necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).
**Usu. focal (wall) involvement; classically leads to berry microaneurysms - ergo the name ''polyarteritis nodosa''.
Image:
*[http://www.immunologyclinic.com/figure.asp?chap=10&fig=14-05d PAN (immunologyclinic.com)].
=Large vessel vasculitides=
The follow section has information specific to the individual types of large vessel vasculitis.
==Giant cell arteritis==
===General===
*[[AKA]] ''temporal arteritis''.
===Clinical===
Features:
*Classic finding: jaw claudication, in a patient older than 50 years.
*Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.
Work-up:
*CRP, ESR, temporal artery biopsy.
Treatment:
*Treat right away with high dose steroids.
**Biopsy is confirmatory.
===Microscopic===
Features:
*Classical: [[granulomas]].
Image(s):
*[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)].
==Takayasu arteritis==
===General===
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Disease of medium/large arteries.
**Classically involves the aortic arch (leading to decreased pulses in the upper limbs).
*Typically in patients <40 yrs old.
*Usually asian.
===Microscopic===
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
*Mononuclear inflammation in media.
*Granulomas, [[giant cells]].
*+/-Patchy necrosis of media.
=Other=
==LAMP-2 vasculitis==
==LAMP-2 vasculitis==
*Associated with pauci-immune necrotizing and crescentic glomerulonephritis.<ref name=pmid19384321>{{cite journal |author=Bosch X, Mirapeix E |title=Vasculitis syndromes: LAMP-2 illuminates pathogenesis of ANCA glomerulonephritis |journal=Nat Rev Nephrol |volume=5 |issue=5 |pages=247–9 |year=2009 |month=May |pmid=19384321 |doi=10.1038/nrneph.2009.51 |url=http://www.nature.com/ki/journal/v76/n1/abs/ki2009123a.html}}</ref>
*Associated with pauci-immune necrotizing and crescentic glomerulonephritis.<ref name=pmid19384321>{{cite journal |author=Bosch X, Mirapeix E |title=Vasculitis syndromes: LAMP-2 illuminates pathogenesis of ANCA glomerulonephritis |journal=Nat Rev Nephrol |volume=5 |issue=5 |pages=247–9 |year=2009 |month=May |pmid=19384321 |doi=10.1038/nrneph.2009.51 |url=http://www.nature.com/ki/journal/v76/n1/abs/ki2009123a.html}}</ref>
Michael
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