Intrathecal rituximab for IgG4-related hypertrophic pachymeningitis

Intrathecal administration of rituximab—an anti-CD20 monoclonal antibody—is emerging as a promising therapeutic strategy for B cell lymphomas of the central nervous system (CNS).1 2 The rationale for administering rituximab directly into the cerebrospinal fluid (CSF) stems from the need to achieve optimal therapeutic concentrations within the intrathecal compartment. Rituximab, in fact, has a high molecular weight and only 0.1%–0.5% of its plasmatic concentrations crosses the 'blood-brain barrier' (BBB) after intravenous infusion.1 In addition, despite inducing prolonged depletion of circulating B lymphocytes, systemic rituximab does not affect malignant B cells in CNS lymphomas.2 Hypertrophic pachymeningitis (HP) is the most frequently encountered CNS manifestation of IgG₄-related disease (IgG₄-RD), a fibroinflammatory condition of unclear aetiology.3 The intrathecal synthesis of IgG₄ in patients with IgG₄-related hypertrophic pachymeningitis(RHP) and the clinical improvement after rituximab in patients with systemic involvement, support a pathogenic role of B lymphocytes.4–6

from #ORL-AlexandrosSfakianakis via ola Kala on Inoreader http://ift.tt/2FLbf9g