Sunday, 26 September 2010

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...