Wegener Granulomatosis
Epidemiology:
Common sites:
- upper respiratory
tract (ear, nose, sinuses, throat)
- lower respiratory
tract (lung)
- kidney
- eye
- skin
- rarely other
organs: heart
Gross features:
- ulcerative lesions
of the nose, palate, or pharynx
Histologic features:
- acute necrotizing granulomas
- upper and lower
respiratory tract
- poorly formed granulomas
- may have ulcers
rimmed by necrotizing granulomas and vasculitis
- necrotizing or granulomatous vasculitis of
small to medium-sized vessels
- lungs, upper
airways most prominently
- surrounded by a
zone of fibroblastic proliferation with giant cells and inflammatory
infiltrate
- undergo
progressive fibrosis and organization
- capillaritis
- alveolar
hemorrhage
- focal necrotizing,
often crescentic, glomerulitis
- similar to Goodpasture and microscopic polyarteritis
- early:
- focal
proliferation and necrosis in the glomeruli
- thrombosis of
isolated glomerular capillary loops
- later/more
severe cases:
- diffuse
necrosis
- fibrin
deposition
- proliferation
- crescent
formation (epithelial, then fibrocellular and
fibrous)
- segmental or
global scarring
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- c-ANCA in serum (up to 95%)
- good marker of
disease activity
- clinical features:
- persistent pneumonitis
with bilateral nodular and cavitary
infiltrates (95%)
- chronic sinusitis (90%)
- mucosal ulcerations of the nasopharynx (75%)
- renal disease (80%)
- hematuria
- rapidly
progressive glomerulonephritis (RPGN) in
severe cases
- skin rashes,
muscle pains, articular involvement, mononeuritis, polyneuritis, fever
- prognosis:
- untreated 80%
mortality in 1 year
References: