Tocilizumab for severe refractory primary central nervous system vasculitis: A center experience

• DOI: 10.1111/ane.13563
• Publication Date: 2021-12-06T06:13:59Z
• Authors: Verónica Cabreira; Leonor Dias; Bruno Fernandes; Ana Aires; Joana Guimarães; Pedro Abreu; Elsa Azevedo
• Keywords: Tocilizumab; Refractory (planetary science); Brain biopsy

Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management unknown given the absence randomized clinical trials.Explore whether tocilizumab, an anti-interleukin-6 monoclonal antibody, effective treatment refractory PCNSV.Patients PCNSV treated tocilizumab in a single tertiary center were reviewed.Three patients identified. In two them, MRI-revealed ischemic lesions. The other presented subcortical hemispheric pseudotumoral lesion. Brain biopsy was inconclusive patients. Due to significant number relapses deterioration despite immunosuppressive drugs, initiated induced long remission period up 44 months. Observed side effects fungic infection, neutropenia thrombocytopenia (both transitory), pulmonary embolism one cases.Tocilizumab might be therapeutic option (Class IV evidence), its efficacy safety. We propose novel pathway diagnosis therapeutics purpose improving diagnosis, monitoring, prognosis this heterogeneous disorder, setting framework future use condition.