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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Wegener's granulomatosis -b- high mag.jpg | |||
| Width = | |||
| Caption = Granulomatosis with polyangiitis. [[H&E stain]]. | |||
| Synonyms = Wegener's granulomatosis (old term) | |||
| Micro = | |||
| Subtypes = | |||
| LMDDx = small vessel vasculitis (inflammatory cells within the vessel wall, vessel wall injury - such as fibrinoid necrosis), granulomas - typically poorly formed | |||
| Stains = | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Staging = | |||
| Site = [[blood vessels]] - see ''[[vasculitides]]'' | |||
| Assdx = renal failure (due to [[rapidly progressive glomerulonephritis]]), [[pulmonary hemorrhage]] | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = epistasis | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = PR3-ANCA (c-ANCA) +ve | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = dependent on presentation - nasal lesion: cocaine use; other causes of [[pulmonary hemorrhage]]; other causes of [[rapidly progressive glomerulonephritis]] | |||
| Tx = | |||
}} | |||
'''Granulomatosis with polyangiitis''', abbreviated '''GPA''', is a type of [[vasculitis]] that typically afflicts the [[lung]]s and [[kidney]]s. | '''Granulomatosis with polyangiitis''', abbreviated '''GPA''', is a type of [[vasculitis]] that typically afflicts the [[lung]]s and [[kidney]]s. | ||
It was previously known as '''Wegener granulomatosis''', abbreviated '''WG'''. | 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== | ==General== | ||
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===Clinical=== | ===Clinical=== | ||
*Epistasis. | *Epistasis. | ||
*Renal failure - | *Renal failure - presents as ''nephritic syndrome''. | ||
**Renal biopsy: crescentic glomerulonephritis ([[AKA]] [[rapidly progressive glomerulonephritis]]). | **Renal biopsy: crescentic glomerulonephritis ([[AKA]] [[rapidly progressive glomerulonephritis]]). | ||
*[[Pulmonary hemorrhage]]. | *[[Pulmonary hemorrhage]]. | ||
Serology: | Serology: | ||
*c-ANCA +ve.<ref name=Ref_TN2005_RH6>{{Ref TN2005|RH6}}</ref> | *PR3-ANCA (c-ANCA) +ve.<ref name=Ref_TN2005_RH6>{{Ref TN2005|RH6}}</ref> | ||
Notes: | Notes: |
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