Granulomatosis with polyangiitis
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Granulomatosis with polyangiitis, abbreviated GPA, is a type of vasculitis that typically afflicts the lungs and kidneys.
| 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 |
| Clin. DDx | dependent on presentation - nasal lesion: cocaine use; other causes of pulmonary hemorrhage; other causes of rapidly progressive glomerulonephritis |
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)
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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.