リウマチ学: 現在の研究
オープンアクセス

概要

ANCA Associated Cerebral Vasculitis: A Rarest Presentation

Richmond Ronald Gomes*

Anti-Neutrophil Cytoplasmic Antibody (ANCA) associated vasculitis (AAV) is a systemic necrotizing inflammation of the small vessels that commonly involves kidney, lung, upper respiratory tract, skin, gastrointestinal and occasionally peripheral nervous system. Central Nervous System (CNS) is less commonly affected(less than 10% of patients) and is generally part of a multi-organ scenario. Angiography (CT angiography, MRA, or digital subtraction angiography) should be performed but can be normal in half of the patients given the involvement of small-sized vessels, which are beyond the detection capacity of the procedure. Biopsy is rarely performed in clinical practice but can help diagnosis. A complete workup including infective and autoimmune serologies as well as cerebrospinal fluid analysis is required, especially in patients with isolated or inaugural CNS manifestations. Finally, in absence of biopsy and/or demonstration of CNS vascular involvement, diagnosis is often probabilistic and relies on the systemic context and the ANCA positivity. Treatment of AAV-related CNS involvement requires combination of high-dose glucocorticoids and an immunosuppressant, mainly cyclophosphamide. Rituximab may be another option in patients with a contraindication for cyclophosphamide.