TY - JOUR T1 - Rituximab for Primary Angiitis of the Central Nervous System: Report of 2 Patients from the French COVAC Cohort and Review of the Literature JF - The Journal of Rheumatology JO - J Rheumatol SP - 2102 LP - 2103 DO - 10.3899/jrheum.130529 VL - 40 IS - 12 AU - HUBERT DE BOYSSON AU - CAROLINE ARQUIZAN AU - LOIC GUILLEVIN AU - CHRISTIAN PAGNOUX Y1 - 2013/12/01 UR - http://www.jrheum.org/content/40/12/2102.abstract N2 - To the Editor:Primary angiitis of the central nervous system (PACNS) is a rare and severe inflammatory disease affecting the vessels of the brain and sometimes spinal cord. Diagnosis relies on (1) a neurologic deficit that remains unexplained after complete examination; (2) CNS vascular abnormalities on conventional cerebral angiography (CCA) or histopathology; and (3) the exclusion of other differential diagnoses such as reversible cerebral vasoconstriction syndrome or systemic vasculitides1,2,3. Neurologic manifestations usually worsen or relapse in the absence of treatment. Corticosteroids are the cornerstone of therapy and with severe disease are often combined with an immunosuppressant, mainly cyclophosphamide (CYC). Few patients are refractory to this treatment and/or relapse. To date, only limited information exists on potential rescue and alternative treatments, including rituximab (RTX)4.We describe 2 adult patients with PACNS who showed improvement with RTX and corticosteroids. In a 42-year-old man, progressive cerebellar ataxia developed over a couple of months; then left hemiparesis and binocular diplopia developed. Brain magnetic resonance imaging (MRI) revealed multiple … Address correspondence to Dr. H. de Boysson, Service de médecine interne, Centre Hospitalier Universitaire de la côte de Nacre, Caen, France. E-mail: hubertdeboysson{at}yahoo.fr ER -