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Granulomatosis with polyangiitis (view source)
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# | '''Granulomatosis with polyangiitis''', also known as '''Wegener granulomatosis''' is a type of [[vasculitis]] that typically afflicts the [[lung]]s and [[kidney]]s. | ||
==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)]. | |||
==See also== | |||
*[[Vasculitides]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Diagnosis]] | |||
[[Category:Cardiovascular pathology]] | |||