- granulomatous necrotizing vasculitis
- triad of
i) upper airways (granuloma) - rhintis (first sign), epistaxis, sinusitis, saddle nose (perforated septum), otitis, mastoiditis
ii) lungs (granuloma and vasculitis) - multiple pulmonary nodule (coin lesions), cavitating lesion with diffuse alveolar changes, presents with hemoptysis
iii) renal (vasculitis)- segmental crescentric necrotizing glomerulonephritis
- c-ANCA (PR3) positive (highly sensitive and specific)
- treatment: IV methylprednisolone, cyclophosphamide
Poor prognostic indicators
- presence and severity of renal involvement
- elderly
- pulmonary hemorrhage
- poor response to therapy at 2 weeks
- high degree of sclerosed glomeruli/interstitial scarring on biopsy
1 comment:
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