Granulomatosis with polyangiitis
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| Granulomatosis with polyangiitis | |
|---|---|
| Diagnosis in short | |
 Granulomatosis with polyangiitis. H&E stain. | |
|
| |
| Synonyms | Wegener's granulomatosis (old term) |
| LM DDx | small vessel vasculitis (inflammatory cells within the vessel wall, vessel wall injury - such as fibrinoid necrosis), granulomas - typically poorly formed |
| Site | blood vessels - see vasculitides |
|
| |
| Associated Dx | renal failure (due to rapidly progressive glomerulonephritis), pulmonary hemorrhage |
| Signs | epistasis |
| Prevalence | uncommon |
| Blood work | PR3-ANCA (c-ANCA) +ve |
Granulomatosis with polyangiitis, abbreviated GPA, is a type of vasculitis that typically afflicts the lungs and kidneys.
It was previously known as Wegener's granulomatosis, abbreviated WG.
It should not be confused with eosinophilic granulomatosis with polyangiitis, previously known as Churg-Strauss syndrome.
General
- Autoimmune.
Clinical
- Epistasis.
- Renal failure - presents as nephritic syndrome.
- Renal biopsy: crescentic glomerulonephritis (AKA rapidly progressive glomerulonephritis).
- Pulmonary hemorrhage.
Serology:
- PR3-ANCA (c-ANCA) +ve.[1]
Notes:
- Pulmonary hemorrhage syndromes:[2]
- 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.
- Granulomas - typically poorly formed.[3]
- Multinucleated giant cells - common. (???)
- Granulomas - typically poorly formed.[3]
- Vessel wall injury.
- Inflammatory cells within the vessel wall.
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
- Wegener's granulomatosis (flickr.com).
- Wegener's granulomatosis - several crappy images (upmc.edu).
- Wegener's granulomatosis - more crappy images (upmc.edu).
- WG - more images (upmc.edu).
See also
References
- ↑ 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.
- ↑ 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.
- ↑ 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.