Table 1

Indications for rituximab in neurology

DisorderIndicationSummary of best evidenceUK usage and funding
Relapsing–remitting multiple sclerosisMaintenance therapy for relapse preventionPositive phase I and II trials and large real-world retrospective studies in Sweden suggest good efficacy, safety and tolerability.7–12Rarely used in UK as there are several licensed disease-modifying therapies. No established funding pathway.
Neuromyelitis optica spectrum disordersMaintenance therapy for relapse preventionPredominantly retrospective case series (of more than 400 patients in total), which consistently show a marked benefit.13Second-line therapy for patients that relapse despite adequate treatment with azathioprine or mycophenolate mofetil in combination with low-dose prednisolone.14 Funded through the UK NMO Service (www.nmouk.nhs.uk).
Autoimmune encephalitis (other than anti-NMDAR)Acute therapyOne large retrospective study and several case reports suggest a benefit but there are no comparative studies of individual immunotherapies.15Consider if there is inadequate response to first-line therapy. Funding is via IPFR to NHSE or through local trust resources.
Anti-NMDAR encephalitisAcute therapyThree retrospective studies suggest a benefit but there are no comparative studies of individual immunotherapies.15–17Commissioned by NHSE as second-line therapy if there is inadequate response to corticosteroids, plasma exchange and intravenous immunoglobulin by 4 weeks from first-line treatment initiation or by 6 weeks from symptom onset.18
Primary angiitis of the CNSAcute therapySmall case series (approximately 10 patients in total).22–24Consider if there is inadequate response to corticosteroids and cyclophosphamide. Funding is via IPFR to NHSE or through local trust resources.
ANCA-associated vasculitisRemission induction and relapsing diseaseTwo randomised controlled trials have shown non-inferiority to cyclophosphamide for remission induction.26 27Licensed and recommended by NICE in combination with corticosteroids as an option for inducing remission of severe disease, when cyclophosphamide has failed, is contraindicated or the patient has not completed their family.28
Stiff-person syndromeTreatment of refractory diseaseCase reports suggest a possible benefit but a single small randomised controlled trial was negative.29–32May consider if there is inadequate response to first-line therapy. Funding is via IPFR to NHSE or through local trust resources.
Immune-mediated peripheral neuropathiesTreatment of refractory diseaseMostly small retrospective series in which benefits are modest.34–50 An uncommon subset of patients with CIDP with antibodies to paranodal proteins may benefit more so (case reports).37 38 Two small randomised controlled trials in anti-MAG neuropathy showed marginal benefits.45 46NHSE will not routinely commission rituximab for refractory CIDP, multifocal motor neuropathy, non-systemic vasculitic neuropathy or anti-MAG neuropathy.33 May consider in exceptional circumstances, particularly in IgG4-mediated disease. Funding is via IPFR to NHSE or through local trust resources.
Myasthenia gravisTreatment of refractory diseaseMostly small retrospective case series. Evidence of benefit is much greater in MuSK-associated myasthenia gravis than AChR-associated myasthenia gravis (for which clinical trials are ongoing).51–55Consider if there is inadequate response to first-line therapy, particularly in MuSK-associated myasthenia gravis. Funding is via IPFR to NHSE or through local trust resources.
  • ANCA, antineutrophil cytoplasmic antibody; CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; CNS, central nervous system; IPFR, individual patient funding request; MAG, myelin-associated glycoprotein; NHSE, National Health Service England; NICE, National Institute for Health and Care Excellence; NMDAR, N-methyl-D-aspartate receptor.