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This article deals with the '''vasculitides''' (singular ''vasculitis''). Vascular disease that is not vasculitides is covered in the article ''[[vascular disease]]''. | [[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]]''. | |||
The histology of normal vessels is dealt with in ''[[Vascular_disease#Normal_blood_vessels|normal blood vessels]]''. | |||
==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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===Grouping by size=== | ===Grouping by size=== | ||
====Small vessel vasculitides==== | ====Small vessel vasculitides==== | ||
=====Definition===== | |||
Small vessel vasculitis = vasculitis of vessels smaller than arteries; affects arterioles, venules, and 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 |month=November |pmid=9366584 |doi=10.1056/NEJM199711203372106 |url=http://www.nejm.org/doi/full/10.1056/NEJM199711203372106}}</ref> | |||
*What is an arteriole? | |||
**There is no histologic definition according to ''Sternberg's Histology for Pathologists''; however, a diameter of <100 micrometers is suggested as a definition.<ref name=Ref_H4P2>{{Ref H4P2|769}}</ref> | |||
=====Types===== | |||
*Predominantly cutaneous vasculitis. | *Predominantly cutaneous vasculitis. | ||
*Henoch-Schoenlein purpura. | *[[Henoch-Schoenlein purpura]]. | ||
*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: | ||
*ANCA = anti-neutrophil cytoplasmic antibodies. | *ANCA = anti-neutrophil cytoplasmic antibodies. | ||
**The terminology has changed as more knowledge has been gained: | |||
***MPO-ANCA = p-ANCA. | |||
***PR3-ANCA = c-ANCA. | |||
====Medium vessel vasculitides<ref name=Ref_PBoD8_512>{{Ref PBoD8|512}}</ref>==== | |||
*[[Polyarteritis nodosa]] (PAN). | |||
*[[Kawasaki disease]]. | |||
====Large vessel vasculitides<ref> | ====Large vessel vasculitides<ref name=Ref_PBoD8_512>{{Ref PBoD8|512}}</ref>==== | ||
*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== | ||
===General=== | ===General=== | ||
*Pathologists often cannot, based on morphology alone, arrive at the definitive diagnosis. | *Pathologists often cannot, based on morphology alone, arrive at the definitive diagnosis. | ||
*The presentation & distribution are more characteristic than the pathology.<ref>URL: [http://www.pathology.ubc.ca/path425/PrincipleofPathophysiology/CirculatoryDisorders/SystemicVasculitisDrBWalker.doc http://www.pathology.ubc.ca/path425/PrincipleofPathophysiology/CirculatoryDisorders/SystemicVasculitisDrBWalker.doc]. Accessed on: 26 November 2010.</ref><ref>URL: [http://www.icapture.ubc.ca/who/who_bios_david_walker.shtml http://www.icapture.ubc.ca/who/who_bios_david_walker.shtml]. Accessed on: 26 November 2010.</ref> | |||
===Microscopic=== | ===Microscopic=== | ||
Features: | 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. | |||
#Vessel injury: | |||
**Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material. | #*Frank fibrinoid [[necrosis]] ''or'' nuclear dust: | ||
***Amorphous = no definite form.<ref>URL: [http://dictionary.weather.net/dictionary/amorphous http://dictionary.weather.net/dictionary/amorphous]. Accessed on: 26 November 2010.</ref> | #**Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material. | ||
**"Nuclear dust" = punctate hyperchromatic material ~ 1 micrometre. | #***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. | |||
*+/-[[RBC extravasation]] - common. | |||
Notes: | |||
*Involvement is usually patchy. | |||
**If there is an inkling of vasculitis... it should prompt [[deeper cuts]]. | |||
====Features to consider==== | |||
#Presence of [[granuloma]]s. | |||
#Type inflammatory cells, i.e. eosinophils, mononuclear cells. | |||
#Size of vessels involved. | |||
#Extent of involvement. | |||
#Acuity (acute vs. subacute vs. chronic vs. acute on chronic). | |||
#*Chronic = thick fibrotic appearing vessels with a small lumen. | |||
====Vasculitis versus neuropathy==== | |||
{| class="wikitable sortable" | |||
! Domain | |||
! Vasculitis | |||
! Neuropathy | |||
|- | |||
|Clinical | |||
|pain, diffuse/<br>patchy distribution | |||
|focal/isolated | |||
|- | |||
|Pathological<br>(inflammatory cells) | |||
|epineurium | |||
|endoneurium | |||
|} | |||
=Small vessel vasculitides= | |||
The follow section has information specific to the individual types of small vessel vasculitis. | |||
==Small vessel leukocytoclastic vasculitis== | |||
*[[AKA]] ''leukocytoclastic vasculitis'', abbreviated ''LCV''. | |||
{{Main|Small vessel leukocytoclastic vasculitis}} | |||
== | ==Microscopic polyangiitis== | ||
===General=== | ===General=== | ||
* | *Classically MPO-ANCA (p-ANCA) +ve. | ||
=== | ===Microscopic=== | ||
Features: | Features - small-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). | ||
*No granulomas. | |||
Images: | |||
* | *[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= | |||
The follow section has information specific to the individual types of medium vessel vasculitis. | |||
== | ==Kawasaki disease== | ||
===General=== | ===General=== | ||
*Medium vessel disease. | |||
* | *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 - 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: | |||
*High dose IV aspirin. | |||
===Microscopic=== | ===Microscopic=== | ||
Features: | Features: | ||
* | *Medium-sized vessels with intramural inflammatory cells. | ||
* | *Vessel destruction, e.g. [[fibrinoid necrosis]] (very pink anucleate arterial wall). | ||
==Polyarteritis nodosa== | ==Polyarteritis nodosa== | ||
*Abbreviated ''PAN''. | |||
===General=== | ===General=== | ||
*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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===Microscopic=== | ===Microscopic=== | ||
Features: | Features - medium-sized vessels with: | ||
*Inflammatory cells (neutrophils, lymphocytes) within the tunica media. | *Inflammatory cells (neutrophils, lymphocytes) within the tunica media. | ||
*Fibroid necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material). | *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== | |||
:''Temporal artery'' redirects here. | |||
*Abbreviated ''GCA''. | |||
*[[AKA]] ''temporal arteritis''. | |||
{{Main|Giant cell arteritis}} | |||
==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. | |||
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=== | |||
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= | ||
=== | ==Aortitis== | ||
* | ===General=== | ||
*Uncommon. | |||
===Gross=== | |||
* | Features: | ||
*Tree bark-like appearance. | |||
Notes: | 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=== | ===Microscopic=== | ||
Features: | Features: | ||
* | *Inflammatory cells. | ||
Subclassification: | |||
*Granulomatous. | |||
*Lymphoplasmacytic pattern. | |||
*Mixed inflammatory. | |||
*Suppurative. | |||
==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> | |||
*Grouped with the ANCA-associated vasculitides.<ref name=pmid19646356>{{cite journal |author=Chen M, Kallenberg CG |title=New advances in the pathogenesis of ANCA-associated vasculitides |journal=Clin. Exp. Rheumatol. |volume=27 |issue=1 Suppl 52 |pages=S108–14 |year=2009 |pmid=19646356 |doi= |url=}}</ref> | |||
=See also= | |||
*[[Cardiovascular pathology]]. | *[[Cardiovascular pathology]]. | ||
*[[Vascular disease]] - covers atherosclerosis, medial cystic degeneration. | *[[Vascular disease]] - covers atherosclerosis, medial cystic degeneration. | ||
*[[Inflammatory skin disorders]]. | |||
*[[Umbilical cord vasculitis]]. | |||
=References= | |||
{{reflist|2}} | {{reflist|2}} | ||
[[Category:Cardiovascular pathology]] | [[Category:Cardiovascular pathology]] | ||
[[Category:Vasculitides]] |
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