Difference between revisions of "Vasculitides"

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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>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>====
====Medium vessel vasculitides<ref name=Ref_PBoD8_512>{{Ref PBoD8|512}}</ref>====
*Polyarteritis nodosa (PAN).
*[[Polyarteritis nodosa]] (PAN).
*Kawasaki disease.
*[[Kawasaki disease]].


====Large vessel vasculitides<ref name=Ref_PBoD8_512>{{Ref PBoD8|512}}</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.
*Presentation & distribution are more characteristic than 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>
*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:<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:  
#*Frank fibrinoid necrosis ''or'' nuclear dust:
#*Frank fibrinoid [[necrosis]] ''or'' nuclear dust:
#**Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material.
#**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>
#***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:
*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
|}


==Giant cell arteritis==
=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===
*[[AKA]] ''temporal arteritis''.
*Classically MPO-ANCA (p-ANCA) +ve.


===Clinical===
===Microscopic===
Features:
Features - small-sized vessels with:
*Classic finding: jaw claudication, in a patient older than 50 years.
*Inflammatory cells (neutrophils, lymphocytes) within the tunica media.
*Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.
*Fibroid necrosis: dead vessel wall - pink anucleate stuff, nuclear debris (black specks of nuclear material).
*No granulomas.


Work-up:
Images:
*CRP, ESR, temporal artery biopsy.
*[http://path.upmc.edu/cases/case260/micro.html Microscopic polyangiitis - several images (upmc.edu)].


Treatment:
==Granulomatosis with polyangiitis==
*Treat right away with high dose steroids.
:Previously known as ''Wegener granulomatosis''.
**Biopsy is confirmatory.
{{Main|Granulomatosis with polyangiitis}}


===Micrograph===
==Eosinophilic granulomatosis with polyangiitis==
Features:
:Previously known as ''Churg-Strauss syndrome'' and ''Churg-Strauss disease''.
*Classical: granulomas.
{{Main|Eosinophilic granulomatosis with polyangiitis}}


Image(s):
=Medium vessel vasculitides=
*[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)].
The follow section has information specific to the individual types of medium vessel vasculitis.


==Takayasu's arteritis==
==Kawasaki disease==
===General===
===General===
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Medium vessel disease.
*Disease of medium/large arteries.
*Classically afflicts the coronary arteries of children - usu. less than 5 years old.
**Classically involves the aortic arch (leading to decreased pulses in the upper limbs).
**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>
*Typically in patients <40 yrs old.  
 
*Usually asian.
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:<ref name=Ref_PBoD538>{{Ref PBoD|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''.
===General===
===General===
*Abbreviated ''PAN''.
*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:
Line 93: Line 165:


===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.


Image: [http://www.immunologyclinic.com/figure.asp?chap=10&fig=14-05d PAN (immunologyclinic.com)].
==Giant cell arteritis==
:''Temporal artery'' redirects here.
*Abbreviated ''GCA''.
*[[AKA]] ''temporal arteritis''.
{{Main|Giant cell arteritis}}


==Wegener's granulomatosis==
==Takayasu arteritis==
===Clinical===
===General===
*Epistasis.
Features:<ref name=Ref_PBoD538>{{Ref PBoD|538}}</ref>
*Renal failure - present as ''nephritic syndrome''.
*Disease of medium/large arteries.
**Renal biopsy: crescentic glomerulonephritis ([[AKA]] rapidly progressive glomerulonephritis).
**Classically involves the aortic arch - leading to decreased pulses in the upper limbs.
*Pulmonary hemorrhage.
*Typically in patients <40 yrs old.  
*Usually Asian.


Serology:
Pathogenesis:
*c-ANCA +ve.<ref>TN05 RH6.</ref>
*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:
*Pulmonary hemorrhage syndromes:<ref name=Ref_PBoD745>{{Ref PBoD|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 name=Ref_PBoD747>{{Ref PBoD|747}}</ref>
*Inflammatory cells.
 
Subclassification:
*Granulomatous.
*Lymphoplasmacytic pattern.
*Mixed inflammatory.
*Suppurative.


Image: [http://www.flickr.com/photos/pulmonary_pathology/3734403695/ Wegener's granulomatosis (flickr.com)].
==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==
=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==
=References=
{{reflist|2}}
{{reflist|2}}


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

Latest revision as of 11:42, 25 November 2016

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 normal blood vessels.

Overview

Most common[1]

  • Polyarteritis nodosa (PAN).
  • Microscopic polyangiitis.
  • Granulomatosis with polyangiitis (Wegener's granulomatosis).
  • Predominantly cutaneous vasculitis.
  • Giant cell arteritis (GCA).

Grouping by size

Small vessel vasculitides

Definition

Small vessel vasculitis = vasculitis of vessels smaller than arteries; affects arterioles, venules, and capillaries.[2]

  • 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.[3]
Types

Notes:

  • 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[4]

Large vessel vasculitides[4]

Grouping by hypersensitivity

Cell-mediated hypersensitivity:[5]

  • 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:[5]

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.[6][7]

Microscopic

Features - both #1 and #2 are required:[8]

  1. Inflammatory cells within the blood vessel wall.
  2. Vessel injury:
    • Frank fibrinoid necrosis or nuclear dust:
      • Fibrinoid necrosis = anucleate amorphous intensely eosinophilic material.
        • Amorphous = no definite form.[9]
      • "Nuclear dust" = punctate hyperchromatic material ~ 1 micrometre.

Notes:

  • Involvement is usually patchy.
    • If there is an inkling of vasculitis... it should prompt deeper cuts.

Features to consider

  1. Presence of granulomas.
  2. Type inflammatory cells, i.e. eosinophils, mononuclear cells.
  3. Size of vessels involved.
  4. Extent of involvement.
  5. Acuity (acute vs. subacute vs. chronic vs. acute on chronic).
    • Chronic = thick fibrotic appearing vessels with a small lumen.

Vasculitis versus neuropathy

Domain Vasculitis Neuropathy
Clinical pain, diffuse/
patchy distribution 		focal/isolated
Pathological
(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 article: Small vessel leukocytoclastic vasculitis

Microscopic polyangiitis

General

  • Classically MPO-ANCA (p-ANCA) +ve.

Microscopic

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:

Granulomatosis with polyangiitis

Previously known as Wegener granulomatosis.
Main article: Granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis

Previously known as Churg-Strauss syndrome and Churg-Strauss disease.
Main article: Eosinophilic granulomatosis with polyangiitis

Medium vessel vasculitides

The follow section has information specific to the individual types of medium vessel vasculitis.

Kawasaki disease

General

  • Medium vessel disease.
  • Classically afflicts the coronary arteries of children - usu. less than 5 years old.
    • May lead to coronary artery aneurysms.[10]

Clinical features - mnemonic Warm CREAM:[11]

  • Warm = fever.
  • Conjunctivitis, non-exudative.
  • Rash, polymorphous.
  • Erythema or edema of hands and feet.
  • Adenopathy, usu. cervical and unilateral.
  • Mucosal manifestations - strawberry tongue, cracked lips.

Treatment:

  • High dose IV aspirin.

Microscopic

Features:

  • Medium-sized vessels with intramural inflammatory cells.
  • Vessel destruction, e.g. fibrinoid necrosis (very pink anucleate arterial wall).

Polyarteritis nodosa

  • Abbreviated PAN.

General

  • Involves small and medium sized vessels.
  • Often - renal vessels, mesenteric vessels.[12]
    • Classically, lung involvement by PAN is considered to be rare, though this may not be entirely true.[13]
  • 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:

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 article: Giant cell arteritis

Takayasu arteritis

General

Features:[14]

  • 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:

Gross

Features:[14]

  • Classically involves the aortic arch.

Microscopic

Features:[14]

  • 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:

  • Several blocks should be submitted.

Image:

Microscopic

Features:

  • Inflammatory cells.

Subclassification:

  • Granulomatous.
  • Lymphoplasmacytic pattern.
  • Mixed inflammatory.
  • Suppurative.

LAMP-2 vasculitis

  • Associated with pauci-immune necrotizing and crescentic glomerulonephritis.[18]
  • Grouped with the ANCA-associated vasculitides.[19]

See also

References

  1. ↑ Yeung, J.C.; Leonard, Blair J. N. (2005). The Toronto Notes 2005 - Review for the MCCQE and Comprehensive Medical Reference (2005 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. RH3. ISBN 978-0968592854.
  2. ↑ Jennette JC, Falk RJ (November 1997). "Small-vessel vasculitis". N. Engl. J. Med. 337 (21): 1512–23. doi:10.1056/NEJM199711203372106. PMID 9366584. http://www.nejm.org/doi/full/10.1056/NEJM199711203372106.
  3. ↑ Sternberg, Stephen S. (1997). Histology for Pathologists (2nd ed.). Lippincott Williams & Wilkins. pp. 769. ISBN 978-0397517183.
  4. ↑ 4.0 4.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 512. ISBN 978-1416031215.
  5. ↑ 5.0 5.1 URL: http://dermind.tripod.com/vasculitis.htm. Accessed on: 30 April 2012.
  6. ↑ URL: http://www.pathology.ubc.ca/path425/PrincipleofPathophysiology/CirculatoryDisorders/SystemicVasculitisDrBWalker.doc. Accessed on: 26 November 2010.
  7. ↑ URL: http://www.icapture.ubc.ca/who/who_bios_david_walker.shtml. Accessed on: 26 November 2010.
  8. ↑ Dillon, MJ.; Eleftheriou, D.; Brogan, PA. (Sep 2010). "Medium-size-vessel vasculitis.". Pediatr Nephrol 25 (9): 1641-52. doi:10.1007/s00467-009-1336-1. PMC 2908435. PMID 19946711. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908435/.
  9. ↑ URL: http://dictionary.weather.net/dictionary/amorphous. Accessed on: 26 November 2010.
  10. ↑ Taubert, KA.; Shulman, ST. (Jun 1999). "Kawasaki disease.". Am Fam Physician 59 (11): 3093-102, 3107-8. PMID 10392592.
  11. ↑ URL: http://www.medicalmnemonics.com/cgi-bin/return_browse.cfm?&system=Other%2FMiscellaneous&discipline=Pathology&browse=1. Accessed on: 14 January 2012.
  12. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 14. ISBN 978-1416002741.
  13. ↑ Matsumoto, T.; Homma, S.; Okada, M.; Kuwabara, N.; Kira, S.; Hoshi, T.; Uekusa, T.; Saiki, S. (Jul 1993). "The lung in polyarteritis nodosa: a pathologic study of 10 cases.". Hum Pathol 24 (7): 717-24. PMID 8100552.
  14. ↑ 14.0 14.1 14.2 Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 538. ISBN 0-7216-0187-1.
  15. ↑ Arnaud, L.; Haroche, J.; Mathian, A.; Gorochov, G.; Amoura, Z. (Nov 2011). "Pathogenesis of Takayasu's arteritis: a 2011 update.". Autoimmun Rev 11 (1): 61-7. doi:10.1016/j.autrev.2011.08.001. PMID 21855656.
  16. ↑ URL: http://dermind.tripod.com/vasculitis.htm. Accessed on: 30 April 2012.
  17. ↑ Vaideeswar, P.. "Syphilitic aortitis: rearing of the ugly head.". Indian J Pathol Microbiol 53 (4): 624-7. doi:10.4103/0377-4929.72002. PMID 21045381.
  18. ↑ Bosch X, Mirapeix E (May 2009). "Vasculitis syndromes: LAMP-2 illuminates pathogenesis of ANCA glomerulonephritis". Nat Rev Nephrol 5 (5): 247–9. doi:10.1038/nrneph.2009.51. PMID 19384321. http://www.nature.com/ki/journal/v76/n1/abs/ki2009123a.html.
  19. ↑ Chen M, Kallenberg CG (2009). "New advances in the pathogenesis of ANCA-associated vasculitides". Clin. Exp. Rheumatol. 27 (1 Suppl 52): S108–14. PMID 19646356.
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