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
Investigations:
- 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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