Giant Cell Arteritis and Spinal Cord Compression: An Overlap Syndrome?

Abstract

We describe 2 patients with spinal cord compression that occurred in the course of biopsy-proven giant cell arteritis (GCA). One case was due to an epidural tumorlike inflammatory lesion, the other to a concentric inflammatory thickening of the meninges. Both patients were highly corticodependent; they had low-titer anti-neutrophil cytoplasmic antibodies but no antimyeloperoxidase or antiproteinase 3 autoantibodies. The diagnosis was established by surgical biopsy. The histological pattern was reminiscent of Wegener granulomatosis. Both patients experienced relapse, despite high doses of corticosteroids, and experienced remission after the introduction of cyclophosphamide. Intravenous immunoglobulin perfusions were added for 1 patient. To our knowledge, spinal cord compression by a spinal pseudotumor or inflammatory meningitis has not been reported in the course of GCA. An overlap syndrome between GCA and Wegener granulomatosis is discussed.