Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica

Neuromyelitis optica (NMO), or Devic's disease, is an aggressive inflammatory disease of the central nervous system that predominantly affects the optic nerves and the spinal cord. The association with other systemic autoimmune diseases and the discovery of the specific biomarker, NMO-immunoglobulin G (IgG), suggests that NMO is autoimmune in origin. The prognosis of NMO is grave, especially in those patients with early and recurrent relapses. We describe successful use of immunoablative cyclophosphamide in halting relapses in a patient with systemic lupus erythematosus-associated NMO who was unresponsive to high-dose oral and intravenous corticosteroids, intravenous immunoglobulin, mycophenolate mofetil, tacrolimus, low-dose daily oral cyclophosphamide and rituximab.