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Vasculitides (view source)

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This article deals with the '''vasculitides''' (singular ''vasculitis'').
[[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>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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===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 (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:
*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 name=Ref_PBoD8_512>{{Ref PBoD8|512}}</ref>====
*[[Giant cell arteritis]] (AKA ''temporal 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==
===General===
*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===
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:
#*Frank fibrinoid [[necrosis]] ''or'' nuclear dust:
#**Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material.
#***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}}


====Large vessel vasculitides<ref>TN05 RH20.</ref>====
==Microscopic polyangiitis==
*Giant cell arteritis (AKA ''temporal arteritis'').
===General===
*Takayasu's arteritis.
*Classically MPO-ANCA (p-ANCA) +ve.


==Giant cell arteritis==
===Microscopic===
*[[AKA]] ''temporal arteritis''.
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}}


===Clinical===
==Eosinophilic granulomatosis with polyangiitis==
Features:
:Previously known as ''Churg-Strauss syndrome'' and ''Churg-Strauss disease''.
*Classic finding: jaw claudication, in a patient older than 50 years.
{{Main|Eosinophilic granulomatosis with polyangiitis}}
*Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.


Work-up:
=Medium vessel vasculitides=
*CRP, ESR, temporal artery biopsy.
The follow section has information specific to the individual types of medium vessel vasculitis.


Treatment:
==Kawasaki disease==
*Treat right away with high dose steroids.
===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>


===Micrograph===
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>
Features:
*'''Warm''' = fever.
*Classical: granulomas.
*'''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.


==Takayasu's arteritis==
Treatment:
General:<ref>PBoD P.538.</ref>
*High dose IV aspirin.
*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===
===Microscopic===
Features:<ref>PBoD P.538.</ref>
Features:
*Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
*Medium-sized vessels with intramural inflammatory cells.
*Mononuclear inflammation in media.
*Vessel destruction, e.g. [[fibrinoid necrosis]] (very pink anucleate arterial wall).
*Granulomas, giant cells.
*+/-Patchy necrosis of media.


==Polyarteritis nodosa==
==Polyarteritis nodosa==
*Abbreviated ''PAN''
*Abbreviated ''PAN''.
===General===
*Involves small and medium sized vessels.
*Involves small and medium sized vessels.
*Often - renal vessels, mesenteric vessels.<ref>Klatt. AOP P.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.
*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}}


==Wegener's granulomatosis==
===Gross===
===Clinical====
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Epistasis.
*Classically involves the aortic arch.
*Renal failure - present as ''nephritic syndrome''.
**Renal biopsy: crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis).
*Pulmonary hemorrhage.


Serology:
===Microscopic===
*c-ANCA +ve.<ref>TN05 RH6.</ref>
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:
*Pulmonary hemorrhage syndromes:<ref>PBoD p.745.</ref>
*Several blocks should be submitted.
**Goodpasture syndrome.
 
**Idiopathic pulmonary hemosiderosis.
Image:
**Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener's granulomatosis).
*[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>
**Systemic lupus erythematosus.


===Microscopic===
===Microscopic===
Features:
Features:
*Granulomas typically poorly formed.<ref>PBoD p.747.</ref>
*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]].
*[[Vascular disease]] - covers atherosclerosis, medial cystic degeneration.
*[[Inflammatory skin disorders]].
*[[Umbilical cord vasculitis]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Cardiovascular pathology]]
[[Category:Cardiovascular pathology]]
[[Category:Vasculitides]]
Michael
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