Granulomatosis with polyangiitis

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Vasculitis of small and medium sized arteries

Presentation

  • Nasal discharge, oral ulcers, sinusitis
  • Dyspnea, cough, sinusitis
  • Renal insufficiency (rapidly progressive glomerulonephritis)
  • Fever, weight loss, fatigue

Findings

  • CXR: Nodular densities and alveolar/pleural opacities
  • C-ANCA +
  • Biopsy of active disease - nasopharyngeal preferred (Dx)

Treatment

  • High-dose corticosteroids
  • Cytotoxic agents