Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 28, 2008
Wegener Granulomatosis
- CNS involvement: 25-50%
- Types of involvement: cranial neuropathy (most common), meningeal (2-8%), cerebral infarctions, vasculitis, paranasal sinuses (including septal erosion and "saddle" nose deformity), orbital & globe, spinal cord, skin, joints, muscles, heart
- Triad: necrotizing granulomas of upper/lower respiratory tracts, necrotizing vasculitis (arteries and veins), and glomerolunephritis
- Antibodies against proteinases found in monocytes and neutrophils (c-ANCA) are highly specific and can be used to monitor disease.
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