MRCP revision battle 21.3: Wegener's Granulomatosis

Wegener's Granulomatosis is a small/medium vessel necrotising granulomatous vasculitis which tends to affect the upper respiratory tract, lungs and kidneys.


90% of cases present with respiratory symptoms.


Associated features to be aware of include:

• blood nasal discharge
• haemoptysis
• saddle-shaped nose

75% get crescentic glomerulonephritis.


cANCA is positive in 90%, pANCA is positive in 25%


CXR may show large shadows.  Pulmonary haemorrhage is also possible.


Treatment is with steroids, cyclophosphamide and possibly co-trimaoxazole to try and reduce risk of PCP.


I guess this is a good opportunity to reconfront a personal demon in the form of a renal battle...