Coexistence of Neuromyelitis Optica Spectrum Disorder and Proliferative Lupus Nephritis: A Rare Case Report

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disease manifesting with transverse myelitis and optic neuritis. Systemic lupus erythematosus (SLE) also commonly affects kidneys and central nervous system. Here, we describe a case of proven NMOSD for almost 16 years with multiple relapses over a period of time, presented to us with acute nephritic syndrome. Her kidney biopsy revealed class 4 lupus nephritis. She responded to steroid, rituximab, and mycophenolate mofetil and achieved complete remission at 3 months. Subsequent relapse at 6 months was initially treated with addition of tacrolimus without success. Later, she dramatically responded to a single dose again. This is one of few cases of coexistence of both diseases from India. Rituximab, MMF, and steroids should be considered as an initial treatment even for proliferative lupus nephritis when NMOSD and SLE coexist.