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

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 

References

Langford, C. and Hoffman, G. Wegener’s granulomatosis Thorax. 1999; 54(7): 629-637. 

Lapraik, C. et al. BSR and BHPR guidelines for the management of adults with ANCA positive vasculitis. Rheumatology (Oxford). 2007; 46(10): 1615-1616. 

Paddock, M., Lynch, C. and Paska, L. Wegener’s granulomatosis in primary care. JRSM Short Rep. 2010. 1(7): 59.