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Vasculitides (view source)

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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>TN05 RH3.</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]] (c-ANCA > p-ANCA).
**[[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - c-ANCA > p-ANCA.
**[[Churg-Strauss syndrome]] (50% ANCA +ve).
**[[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss syndrome) - 50% ANCA +ve.
**[[Microscopic polyangiitis]] (usually p-ANCA).
**[[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>URL: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908435/ http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908435/]. Accessed on: 26 November 2010.</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 vs. neuropathy====
====Vasculitis versus neuropathy====
{| class="wikitable"
{| class="wikitable sortable"
| |
! Domain
|'''Vasculitis'''
! Vasculitis
|'''Neuropathy'''
! 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}}
===General===
*Most common cutaneous vasculitis.<ref name=pmid18948765>{{Cite journal  | last1 = Brinster | first1 = NK. | title = Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II). | journal = Adv Anat Pathol | volume = 15 | issue = 6 | pages = 350-69 | month = Nov | year = 2008 | doi = 10.1097/PAP.0b013e31818b1ac6 | PMID = 18948765 }}</ref>
 
Clinical:
*Palpable purpura, usu. lower extremity.
 
===Microscopic===
Features:<ref name=pmid18948765/>
*Small upper dermis vessels with:
*#Neutrophils.
*#*Fragmentation of neutrophils (leukocytoclasia).
*#Vessel damage: fibrin deposition (bright pink acellular stuff).
 
Has a very broad DDx:<ref name=pmid18948765/>
#Infectious:
#*Bacterial.
#*Viral.
#*Fungal.
#Vasculitic disorders:
#*ANCA mediated vasculitides:
#**[[Wegener granulomatosis]].
#**[[Churg-Strauss syndrome]].
#*[[Henoch–Schönlein purpura]].<ref name=pmid9713395>{{Cite journal  | last1 = Kraft | first1 = DM. | last2 = Mckee | first2 = D. | last3 = Scott | first3 = C. | title = Henoch-Schönlein purpura: a review. | journal = Am Fam Physician | volume = 58 | issue = 2 | pages = 405-8, 411 | month = Aug | year = 1998 | doi =  | PMID = 9713395 }}</ref>
#*Urticarial vasculitis.
#Other:
#*Connective tissue disease.
#*Cryoglobulinemia - may be due to [[multiple myeloma]], [[hepatitis C]]; have intravascular thrombi.
#*Paraneoplastic.
#*Drugs.
 
Image:
*[http://www.ancavasculitis.com/biopsy.php LCA (ancavasculitis.com)].
*[http://path.upmc.edu/cases/case189.html LCA of the GI tract - several images (upmc.edu)].
 
===Stains===
*PAS - look for fungus.


==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)].


==Wegener granulomatosis==
==Granulomatosis with polyangiitis==
*Abbreviated ''WG''.
:Previously known as ''Wegener granulomatosis''.
*[[AKA]] ''granulomatosis with polyangiitis''.
{{Main|Granulomatosis with polyangiitis}}
===General===
*Autoimmune.
 
====Clinical====
*Epistasis.
*Renal failure - present as ''nephritic syndrome''.
**Renal biopsy: crescentic glomerulonephritis ([[AKA]] [[rapidly progressive glomerulonephritis]]).
*Pulmonary hemorrhage.
 
Serology:
*c-ANCA +ve.<ref>TN05 RH6.</ref>
 
Notes:
*Pulmonary hemorrhage syndromes:<ref name=Ref_PBoD745>{{Ref PBoD|745}}</ref>
**[[Goodpasture syndrome]].
**Idiopathic pulmonary hemosiderosis.
**Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener granulomatosis).
**[[Systemic lupus erythematosus]].
 
===Microscopic===
Features:
*Small vessel vasculitis:
*#Inflammatory cells within the vessel wall.
*#*[[Granuloma]]s - typically poorly formed.<ref name=Ref_PBoD747>{{Ref PBoD|747}}</ref>
*#**Multinucleated giant cells - common. (???)
*#Vessel wall injury.
*#*[[Fibrinoid necrosis]].
 
Notes:
*In the lung, the [[granuloma]]s tend to be centrilobular, as the artery travels with the airway and is centrilobular.
**It may difficult to find small blood vessels in affected portions of lung.
 
Images:
*www:
**[http://www.flickr.com/photos/pulmonary_pathology/3734403695/ Wegener's granulomatosis (flickr.com)].
**[http://path.upmc.edu/cases/case269.html Wegener's granulomatosis - several crappy images (upmc.edu)].
**[http://path.upmc.edu/cases/case660.html Wegener's granulomatosis - more crappy images (upmc.edu)].
**[http://path.upmc.edu/cases/case675.html WG - more images (upmc.edu)].
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Wegener%27s_granulomatosis_-b-_intermed_mag.jpg WG - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Wegener%27s_granulomatosis_-b-_very_high_mag.jpg WG - fibrinoid necrosis - very high mag. (WC)].
 
==Churg-Strauss syndrome==
*AKA ''allergic granulomatous angiitis'',<ref name=emedicine333492>[http://emedicine.medscape.com/article/333492-overview http://emedicine.medscape.com/article/333492-overview]</ref>, [[AKA]] Churg-Strauss disease.
 
===General===
Defining features - memory device ''GAFE'':
*'''G'''ranulomata.
*'''A'''sthma.
*'''F'''ever.
*'''E'''osinophilia.
 
Notes:
*Similar to ''[[Wegener's granulomatosis]]'' (classically c-ANCA +ve) and ''microscopic polyangiitis''.<ref>URL: [http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]. Accessed on: 22 January 2011.</ref>
 
===Microscopic===
Features:
*Small vessel [[vasculitis]].
*Abundant eosinophils.
*[[Granuloma]]s.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Churg-Strauss_syndrome_-_high_mag.jpg CSS - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Churg-Strauss_syndrome_-_very_high_mag.jpg CSS - very high mag. (WC)].


DDx:
==Eosinophilic granulomatosis with polyangiitis==
*Eosinophilic vasculitis associated with a [[connective tissue disease]].<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>
: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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*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''.
*Abbreviated ''GCA''.
*[[AKA]] ''temporal arteritis''.
*[[AKA]] ''temporal arteritis''.
===General===
{{Main|Giant cell arteritis}}
*Classically afflicts the ''temporal artery''.
 
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:
*Artery with intramural inflammatory cells.
**Classically [[granuloma|granulomatous inflammation]].
***Granulomas not required for the diagnosis!
*Destruction of arterial wall, e.g. fibrinoid necrosis (pink anucleate arterial wall).
 
Image(s):
*[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)].
*[http://path.upmc.edu/cases/case646.html GCA - several images (upmc.edu)].


==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 (leading to decreased pulses in the upper limbs).
**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 asian.
*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]]
Michael
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