Anti-neutrophil cytoplasmic antibody (ANCA)-associated granulomatosis vasculitis (AAGV) is a systemic necrotizing vasculitis that affects smalland medium-sized vessels with associated granuloma. It involves mainly the upper and lower respiratory tract and the kidneys. Other organs are less frequently affected (skin, joints, heart, central nervous system, and eyes). Neurological signs are observed in 20–50% of cases [1, 2]. We report a patient with biopsyproven AAGV with positive c-ANCA who had osseous and meningeal spinal localization. A 53-year-old man was referred in March 2003 to the internal medicine department because of persistent fever and dorsal and lumbar pain. Since 2001, he had systemic AAGV with rhinitis and mononeuritis. The c-ANCA test was positive. Steroids led to remission. In December 2002, the vasculitis relapsed and he was treated with steroids and intravenous cyclophosphamide. His temperature was 39.5 C and physical examination was normal without meningeal syndrome. Hemoglobin was 9.6 g/dL, platelet count was 480 g/L, leukocytes were 12 g/L (80% polymorphonuclear neutrophils), C-reactive protein (CRP) was 232 mg/L, serum creatinine was 80 lmol/L, and proteinuria was 2–3 g/L. c-ANCAs were detected by indirect immunofluorescence. Specificity against proteinase-3 (PR-3) and a level of 66 U (normal value \3 U) were determined by an enzyme-linked immunosorbent assay. Blood cultures were negative. Chest X-ray, spinal X-ray, technetium scintigraphy, echocardiography, and thoracic and abdominal computed tomography (CT) scans were normal. The patient refused kidney biopsy. Pulmonary fibroscopy, bone marrow, and muscle biopsies were negative. A spinal CT scan disclosed a prevertebral mass extending from vertebrae T12–L2. Spinal cord magnetic resonance imaging (MRI) disclosed epidural thickening with regard to vertebrae T12–L2 (hypertrophic spinal pachymeningitis) with a T2-weighted hypersignal (Fig. 1) and T1 enhancement with gadolinium. The lesion infiltrated soft tissues, but there was no vertebral nor disk destruction. Percutaneous biopsy of the T12 vertebra did not show necrotic lesions nor altered neutrophil infiltration, while cultures were negative. Empirical treatment with isoniazid, ethambutol, rifampicin, and ciprofloxacin did not improve clinical and biological signs. The brain CT scan and MRI were normal without hypertrophic cranial pachymeningitis. Cerebrospinal fluid analysis revealed 1.5 g/L proteins, 32/lL leucocytes with 84% neutrophils. Microbiological cultures were sterile. A surgical biopsy of the T12 vertebra disclosed granulomatous vasculitis with necrosis, which was indicative of AAGV, with fibrosis, inflammatory infiltration with polymorphonuclear cells, isolated giant cells independent of vessel walls, and some foci of necrosis (Fig. 2). The patient was treated with three pulses (1 g/day) of IV methylprednisolone, followed by 1 mg/kg/day prednisone in combination with 2 mg/kg/day oral cyclophosphamide for 1 year, followed by 2 g/day C. Durant (&) A. Masseau Hopital Saint Louis, Paris, France e-mail: cecile.durant@sls.aphp.fr