Vasculitides: Difference between revisions

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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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*[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====
<gallery>
Image:Wegener's granulomatosis - intermed mag.jpg | WG - intermed. mag. (WC)
Image:Wegener's granulomatosis - very high mag.jpg | WG - very high mag. (WC)
Image:Wegener's granulomatosis -b- intermed mag.jpg | WG - intermed. mag. (WC)
Image:Wegener's granulomatosis -b- high mag.jpg | WG - high mag. (WC)
Image:Wegener's granulomatosis -b- very high mag.jpg | WG - very high mag. (WC)
</gallery>
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)].
 
==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:
*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.


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}}


====Images====
<gallery>
Image:Churg-Strauss_syndrome_-_high_mag.jpg |CSS - high mag. (WC)
Image:Churg-Strauss_syndrome_-_very_high_mag.jpg |CSS - very high mag. (WC)
</gallery>
=Medium vessel vasculitides=
=Medium vessel vasculitides=
The follow section has information specific to the individual types of medium vessel vasculitis.
The follow section has information specific to the individual types of medium vessel vasculitis.
Retrieved from "https://librepathology.org/wiki/Vasculitides"