Difference between revisions of "Vasculitides"

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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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*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.
*Giant cell arteritis.
*Takayasu arteritis.
*Takayasu arteritis.
*Churg-Strauss disease.
*Eosinophilic granulomatosis with polyangiitis (Churg-Strauss disease).
*Wegener’s granulomatosis.
*Granulomatosis with polyangiitis (Wegener’s granulomatosis).


Note:
Note:
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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]], e.g. [[mixed connective tissue disease]], [[SLE]], [[rheumatoid arthritis]].
#*[[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 name=Ref_TN2005_RH6>{{Ref TN2005|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==
*Abbreviated ''CSS''.
*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:
==Eosinophilic granulomatosis with polyangiitis==
*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>
:Previously known as ''Churg-Strauss syndrome'' and ''Churg-Strauss disease''.
 
{{Main|Eosinophilic granulomatosis with polyangiitis}}
===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 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>


=Medium vessel vasculitides=
=Medium vessel vasculitides=
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*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.
**ESR normal (>50 years old): <20 mm/hr males, <30 mm/hr females.<ref>URL: [http://www.nlm.nih.gov/medlineplus/ency/article/003638.htm http://www.nlm.nih.gov/medlineplus/ency/article/003638.htm]. Accessed on: 17 August 2012.</ref>
 
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)].
 
===Sign out===
====Negative====
<pre>
TEMPORAL ARTERY, LEFT, BIOPSY:
- MEDIUM SIZE ARTERY WITHOUT PATHOLOGIC DIAGNOSIS, SEE COMMENT.
 
COMMENT:
A negative biopsy does not rule out the possibility of giant cell (temporal)
arteritis, as this may be a focal disorder. The clinical management is
dependent upon the clinical impression.
</pre>


==Takayasu arteritis==
==Takayasu arteritis==
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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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