We describe 2 cases and review the literature on the spectrum of clinical manifestations associated with meningeal involvement in patients with Wegener granulomatosis (WG). A 31-year-old man and a 60-year-old woman with WG in complete clinical remission developed severe chronic headaches. No inflammatory activity was detectable at sites of previous disease activity, and nonspecific markers of inflammation were within normal limits. However, both patients had persistently elevated titers of antineutrophil cytoplasmic antibodies (cytoplasmic staining pattern). Cerebrospinal fluid examinations showed no abnormalities that suggested inflammation, infection, or malignancy. Head magnetic resonance imaging with gadolinium contrast revealed enhancement of the dura in both patients, and a meningeal biopsy in 1 patient confirmed active WG. Both patients' symptoms resolved after reinstitution of aggressive immunosuppressive therapy.
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