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[[Image:Churg-Strauss syndrome - high mag.jpg|thumb|right|[[Micrograph]] showing a vasculitis. [[H&E stain]].]] | |||
This article deals with the '''vasculitides''' (singular ''vasculitis''). Vascular disease that is not vasculitides is covered in the article ''[[vascular disease]]''. | This article deals with the '''vasculitides''' (singular ''vasculitis''). Vascular disease that is not vasculitides is covered in the article ''[[vascular disease]]''. | ||
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==Overview== | ==Overview== | ||
===Most common<ref> | ===Most common<ref>{{Ref TN2005 |RH3}}</ref>=== | ||
*Polyarteritis nodosa (PAN). | *Polyarteritis nodosa (PAN). | ||
*Microscopic polyangiitis. | *Microscopic polyangiitis. | ||
*Wegener's granulomatosis. | *Granulomatosis with polyangiitis (Wegener's granulomatosis). | ||
*Predominantly cutaneous vasculitis. | *Predominantly cutaneous vasculitis. | ||
*Giant cell arteritis (GCA). | *Giant cell arteritis (GCA). | ||
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*Essential cryoglobulinemic vasculitis. | *Essential cryoglobulinemic vasculitis. | ||
*ANCA-associated: | *ANCA-associated: | ||
**[[Wegener's granulomatosis | **[[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - c-ANCA > p-ANCA. | ||
**[[Churg-Strauss syndrome | **[[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss syndrome) - 50% ANCA +ve. | ||
**[[Microscopic polyangiitis]] | **[[Microscopic polyangiitis]] - usually p-ANCA. | ||
Notes: | Notes: | ||
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*[[Giant cell arteritis]] (AKA ''temporal arteritis''). | *[[Giant cell arteritis]] (AKA ''temporal arteritis''). | ||
*[[Takayasu's arteritis]]. | *[[Takayasu's arteritis]]. | ||
===Grouping by hypersensitivity=== | |||
Cell-mediated [[hypersensitivity]]:<ref name=indian_guy>URL: [http://dermind.tripod.com/vasculitis.htm http://dermind.tripod.com/vasculitis.htm]. Accessed on: 30 April 2012.</ref> | |||
*Giant cell arteritis. | |||
*Takayasu arteritis. | |||
*Eosinophilic granulomatosis with polyangiitis (Churg-Strauss disease). | |||
*Granulomatosis with polyangiitis (Wegener’s granulomatosis). | |||
Note: | |||
*All have granulomas. | |||
[[Immune complex mediated hypersensitivity]]:<ref name=indian_guy>URL: [http://dermind.tripod.com/vasculitis.htm http://dermind.tripod.com/vasculitis.htm]. Accessed on: 30 April 2012.</ref> | |||
*Polyarteritis nodosa. | |||
*Microscopic polyangiitis.{{fact}} | |||
*Leukocytoclastic vasculitis.{{fact}} | |||
**Henoch-Schonlein purpura. | |||
==Pathologist's role in the diagnosis of vasculitis== | ==Pathologist's role in the diagnosis of vasculitis== | ||
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===Microscopic=== | ===Microscopic=== | ||
Features:<ref> | Features - both #1 and #2 are required:<ref name=pmid19946711>{{Cite journal | last1 = Dillon | first1 = MJ. | last2 = Eleftheriou | first2 = D. | last3 = Brogan | first3 = PA. | title = Medium-size-vessel vasculitis. | journal = Pediatr Nephrol | volume = 25 | issue = 9 | pages = 1641-52 | month = Sep | year = 2010 | doi = 10.1007/s00467-009-1336-1 | PMID = 19946711 |PMC = 2908435 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908435/}}</ref> | ||
#Inflammatory cells within the blood vessel wall. | #Inflammatory cells within the blood vessel wall. | ||
#Vessel injury: | #Vessel injury: | ||
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#***Amorphous = no definite form.<ref>URL: [http://dictionary.weather.net/dictionary/amorphous http://dictionary.weather.net/dictionary/amorphous]. Accessed on: 26 November 2010.</ref> | #***Amorphous = no definite form.<ref>URL: [http://dictionary.weather.net/dictionary/amorphous http://dictionary.weather.net/dictionary/amorphous]. Accessed on: 26 November 2010.</ref> | ||
#**"Nuclear dust" = punctate hyperchromatic material ~ 1 micrometre. | #**"Nuclear dust" = punctate hyperchromatic material ~ 1 micrometre. | ||
*+/-[[RBC extravasation]] - common. | |||
Notes: | Notes: | ||
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#*Chronic = thick fibrotic appearing vessels with a small lumen. | #*Chronic = thick fibrotic appearing vessels with a small lumen. | ||
====Vasculitis | ====Vasculitis versus neuropathy==== | ||
{| class="wikitable" | {| class="wikitable sortable" | ||
! Domain | |||
! Vasculitis | |||
! Neuropathy | |||
|- | |- | ||
|Clinical | |Clinical | ||
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==Small vessel leukocytoclastic vasculitis== | ==Small vessel leukocytoclastic vasculitis== | ||
*[[AKA]] ''leukocytoclastic vasculitis'', abbreviated ''LCV''. | *[[AKA]] ''leukocytoclastic vasculitis'', abbreviated ''LCV''. | ||
{{Main|Small vessel leukocytoclastic vasculitis}} | |||
==Microscopic polyangiitis== | ==Microscopic polyangiitis== | ||
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*[http://path.upmc.edu/cases/case260/micro.html Microscopic polyangiitis - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case260/micro.html Microscopic polyangiitis - several images (upmc.edu)]. | ||
== | ==Granulomatosis with polyangiitis== | ||
:Previously known as ''Wegener granulomatosis''. | |||
{{Main|Granulomatosis with polyangiitis}} | |||
== | ==Eosinophilic granulomatosis with polyangiitis== | ||
:Previously known as ''Churg-Strauss syndrome'' and ''Churg-Strauss disease''. | |||
{{Main|Eosinophilic granulomatosis with polyangiitis}} | |||
=Medium vessel vasculitides= | =Medium vessel vasculitides= | ||
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===General=== | ===General=== | ||
*Medium vessel disease. | *Medium vessel disease. | ||
*Classically afflicts the coronary arteries of children. | *Classically afflicts the coronary arteries of children - usu. less than 5 years old. | ||
**May lead to coronary artery aneurysms.<ref>{{Cite journal | last1 = Taubert | first1 = KA. | last2 = Shulman | first2 = ST. | title = Kawasaki disease. | journal = Am Fam Physician | volume = 59 | issue = 11 | pages = 3093-102, 3107-8 | month = Jun | year = 1999 | doi = | PMID = 10392592 |URL = http://www.aafp.org/afp/1999/0601/p3093.html}}</ref> | |||
Clinical features | Clinical features - mnemonic ''Warm CREAM'':<ref>URL: [http://www.medicalmnemonics.com/cgi-bin/return_browse.cfm?&system=Other%2FMiscellaneous&discipline=Pathology&browse=1 http://www.medicalmnemonics.com/cgi-bin/return_browse.cfm?&system=Other%2FMiscellaneous&discipline=Pathology&browse=1]. Accessed on: 14 January 2012.</ref> | ||
* | *'''Warm''' = fever. | ||
* | *'''C'''onjunctivitis, non-exudative. | ||
* | *'''R'''ash, polymorphous. | ||
* | *'''E'''rythema or edema of hands and feet. | ||
* | *'''A'''denopathy, usu. cervical and unilateral. | ||
* | *'''M'''ucosal manifestations - strawberry tongue, cracked lips. | ||
Treatment: | Treatment: | ||
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*Involves small and medium sized vessels. | *Involves small and medium sized vessels. | ||
*Often - renal vessels, mesenteric vessels.<ref name=Ref_Klatt14>{{Ref Klatt|14}}</ref> | *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. | **Classically, [[lung]] involvement by PAN is considered to be rare, though this may not be entirely true.<ref name=pmid8100552>{{Cite journal | last1 = Matsumoto | first1 = T. | last2 = Homma | first2 = S. | last3 = Okada | first3 = M. | last4 = Kuwabara | first4 = N. | last5 = Kira | first5 = S. | last6 = Hoshi | first6 = T. | last7 = Uekusa | first7 = T. | last8 = Saiki | first8 = S. | title = The lung in polyarteritis nodosa: a pathologic study of 10 cases. | journal = Hum Pathol | volume = 24 | issue = 7 | pages = 717-24 | month = Jul | year = 1993 | doi = | PMID = 8100552 }}</ref> | ||
*Strong association with ''[[hepatitis B]]'' (see [[medical liver diseases]]); ~1/3 of patients with PAN have HBV. | |||
Serology: | Serology: | ||
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==Giant cell arteritis== | ==Giant cell arteritis== | ||
:''Temporal artery'' redirects here. | |||
*Abbreviated ''GCA''. | |||
*[[AKA]] ''temporal arteritis''. | *[[AKA]] ''temporal arteritis''. | ||
{{Main|Giant cell arteritis}} | |||
==Takayasu arteritis== | ==Takayasu arteritis== | ||
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Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref> | Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref> | ||
*Disease of medium/large arteries. | *Disease of medium/large arteries. | ||
**Classically involves the aortic arch | **Classically involves the aortic arch - leading to decreased pulses in the upper limbs. | ||
*Typically in patients <40 yrs old. | *Typically in patients <40 yrs old. | ||
*Usually | *Usually Asian. | ||
Pathogenesis: | |||
*Cell-mediated hypersensitivity.<ref name=pmid21855656>{{Cite journal | last1 = Arnaud | first1 = L. | last2 = Haroche | first2 = J. | last3 = Mathian | first3 = A. | last4 = Gorochov | first4 = G. | last5 = Amoura | first5 = Z. | title = Pathogenesis of Takayasu's arteritis: a 2011 update. | journal = Autoimmun Rev | volume = 11 | issue = 1 | pages = 61-7 | month = Nov | year = 2011 | doi = 10.1016/j.autrev.2011.08.001 | PMID = 21855656 }}</ref><ref>URL: [http://dermind.tripod.com/vasculitis.htm http://dermind.tripod.com/vasculitis.htm]. Accessed on: 30 April 2012.</ref>{{fact}} | |||
===Gross=== | |||
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref> | |||
*Classically involves the aortic arch. | |||
===Microscopic=== | ===Microscopic=== | ||
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=Other= | =Other= | ||
==Aortitis== | |||
===General=== | |||
*Uncommon. | |||
===Gross=== | |||
Features: | |||
*Tree bark-like appearance. | |||
Notes: | |||
*Several blocks should be submitted. | |||
Image: | |||
*[http://www.ijpmonline.org/viewimage.asp?img=IndianJPatholMicrobiol_2010_53_4_624_72002_f4.jpg Tree barking (ijpmonline.org)].<ref name=pmid21045381>{{Cite journal | last1 = Vaideeswar | first1 = P. | title = Syphilitic aortitis: rearing of the ugly head. | journal = Indian J Pathol Microbiol | volume = 53 | issue = 4 | pages = 624-7 | month = | year = | doi = 10.4103/0377-4929.72002 | PMID = 21045381 }}</ref> | |||
===Microscopic=== | |||
Features: | |||
*Inflammatory cells. | |||
Subclassification: | |||
*Granulomatous. | |||
*Lymphoplasmacytic pattern. | |||
*Mixed inflammatory. | |||
*Suppurative. | |||
==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> | ||
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*[[Vascular disease]] - covers atherosclerosis, medial cystic degeneration. | *[[Vascular disease]] - covers atherosclerosis, medial cystic degeneration. | ||
*[[Inflammatory skin disorders]]. | *[[Inflammatory skin disorders]]. | ||
*[[Umbilical cord vasculitis]]. | |||
=References= | =References= | ||
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[[Category:Cardiovascular pathology]] | [[Category:Cardiovascular pathology]] | ||
[[Category:Vasculitides]] |
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