Background GPA, a small-sized–vessel vasculitis, commonly involves ear, nose & throat (ENT), lungs and kidneys, and rarely, the CNS.
Objectives The presentation, management and outcome of GPA CNS involvement were evaluated.
Methods We retrospectively reviewed the charts of 16 patients (12 men) with: GPA satisfying ACR and/or Chapel Hill criteria; and, after excluding other causes, GPACNS involvement manifesting as pachymeningitis, meningitis, stroke, spinal cord involvement or hypophyseal involvement.
Results Mean respective ages at GPA diagnosis and onset of CNS involvement were 43 and 47 years. The latter was present in 9 (56%) patients at GPA diagnosis, and appeared in the 7 others after a median follow-up of 24 months. Headache was the main symptom (67%), with motor and sensory impairments noted in 33 and 27%, respectively. CNS involvements were: pachymeningitis (n=8: 7 cranial and 1 spinal cord), ischemic (n=4) or hemorrhagic stroke (n=2), cerebral vasculitis (n=2), and/or hypophyseal involvement (n=2). Extra-CNS manifestations included ENT (75%), lungs (60%), peripheral nerve(s) (40%) and kidneys (33%). ANCA detected in 12/16 (75%) patients had PR3 (n=7) or MPO (n=5) specificity.
Induction therapy comprised corticosteroids (CS, 100%) and IV (69%) or oral cyclophosphamide (CYC, 25%) or rituximab (6%). Maintenance therapy consisted of CS (100%) and azathioprine (63%), or methotrexate or rituximab (13% each).
CNS involvement responded clinically in 12/16 (75%). Relapsing and/or refractory CNS GPA in 7 patientswas treated with IV CYC or rituximab (43% each) or oral CYC (14%). No patient died during follow-up, but 64% had persistent neurologicalsequelae.
Conclusions Our series highlights the heterogeneity of CNS involvement in GPA. Despite initial severe disease, conventional therapy obtained clinical improvement in 75% of the patients. Rituximab should be evaluated for refractory and/or relapsing CNS GPA.
Disclosure of Interest None Declared