Wegener’s granulomatosis

Wegener’s granulomatosis is a small and medium vessel necrotizing granulomatous vasculitis which predominantly affects the upper airways, lungs and kidneys but can affect any organ system. 

Possible features include:
  • Respiratory symptoms – 90%
    • Cough 
    • Pleuritis 
    • Haemoptysis 
    • Rhinorrhoea 
    • Epistaxis 
  • Sinusitis 
  • Subglottic stenosis -20% 
  • Saddle-shaped nose 
  • Perforation of the nasal septum 
  • Glomerulonephritis – develops in 80% at some point

  • Biopsy 
    • Necrotising granulomatous vasculitis with neutrophilic infiltrates
  • CXR 
    • Bilateral nodular infiltrates 
    • Alveolar haemorrhage 
  • ANCA
    • c-ANCA – positive in 90%
    • p-ANCA – positive in 10% 

Treatment: steroids and cyclophosphamide, with co-trimoxazole to decrease opportunitistic infections 

5 year survival with treatment is around 76% 

Small print gem: Long-term treatment with cyclophosphamide is associated with increased risk of bladder cancer and myelodysplasia 

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