• A C Desbois, N Shor, C Chapelon, E Maillart, V Touitou, P Cacoub, and D Saadoun.
• Sorbonne universités, AP-HP, groupe hospitalier Pitié-Salpêtrière, département de médecine interne et immunologie clinique, 75013 Paris, France; Centre national de référence maladies auto-immunes et systémiques rares, centre national de référence maladies auto inflammatoires rares et amylose inflammatoire, Inserm, UMR S 959, Immunology-Immunopathology- Immunotherapy (I3), 75005 Paris, France. Electronic address: anneclaire.desbois@aphp.fr.
• Rev Med Interne. 2023 Mar 1; 44 (3): 123132123-132.

AbstractNeurosarcoidosis (NS) is a rare but severe form of sarcoidosis. NS is associated with significant morbidity and mortality. Mortality is about 10% at 10 years with more than 30% of patients who have a significant disability. The most frequent features are cranial neuropathy (the facial and optic nerve most commonly affected), cranial parenchymal lesions, meningitis, spinal corn abnormalities (20-30%) and more rarely peripheral neuropathy (approximately 10-15%). The challenge of diagnosis is to eliminate other diagnoses. Atypical presentations should make to discuss the need for cerebral biopsy in order to highlight the presence of granulomatous lesions while eliminating alternative diagnosis. Therapeutic management is based on corticosteroid therapy and immunomodulators. There are no comparative prospective study to allow us to define the first-line immunosuppressive treatment and the therapeutic strategy in refractory patients. Conventional immunosuppressants such as methotrexate, mycophenolate mofetil and cyclophosphamide are commonly used. Data on the efficacy of anti-TNFα (including infliximab) in refractory and/or severe forms are increasing during the last ten years. Additional data is necessary to assess their interest in first line in patients with severe involvement and a significant risk of relapse.Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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