Difference between revisions of "Granulomatosis with polyangiitis"

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#redirect [[Vasculitides#Wegener_granulomatosis]]
'''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]]

Revision as of 16:51, 31 August 2016

Granulomatosis with polyangiitis, also known as Wegener granulomatosis is a type of vasculitis that typically afflicts the lungs and kidneys.

General

  • Autoimmune.

Clinical

Serology:

Notes:

Microscopic

Features:

  • Small vessel vasculitis:
    1. Inflammatory cells within the vessel wall.
      • Granulomas - typically poorly formed.[3]
        • Multinucleated giant cells - common. (???)
    2. Vessel wall injury.

Notes:

  • In the lung, the granulomas 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

  • WG - intermed. mag. (WC)

  • WG - very high mag. (WC)

  • WG - intermed. mag. (WC)

  • WG - high mag. (WC)

  • WG - very high mag. (WC)

www

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. RH6. ISBN 978-0968592854.
  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. 745. ISBN 0-7216-0187-1.
  3. ↑ 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. 747. ISBN 0-7216-0187-1.
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