Crescentic glomerulonephritis in a patient with neuromyelitis optica (Devic´s syndrom)

Abstract

Neuromyelitis optica (NMO) or Devic´s syndrome is an inflammatory, demyelinating disease of the central nervous system leading to optical neuritis and myelitis. Etiology of NMO is unknown, although autoimmune disorders involving IgG autoantibodies specific for aquaporin 4 are suspected. These autoantibodies are associated with 73% sensitivity 93% specificity for NMO helping to distinguish NMO from other demyelinating disorders. Some cases of NMO are reported in association with systemic lupus erythematosus and Sjögren`s syndrome. Here we report a patient presenting with NMO and concomitantly occurring rapid progressive crescentic intra-and extracapillary crescentic glomerulonephritis.