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Published on: January 29, 2014
Hyper IgM syndrome and complement Clq deficiency in an individual with systemic lupus erythematosus-like disease
1Department of Paediatrics, Fujita Health University, Aichi, Japan. itsuge@fujita-hu.ac.jp
Abstract:
Many immunedeficiency syndromes are associated with autoimmune disorders. We here report on a girl with a systemic lupus erythematosus-like disease who suffered from both hyperimmunoglobulin M syndrome (HIGMS) and C1q deficiency. Despite severe central nervous system-lupus like disease, probably due to C1q deficiency, kidney function was relatively spared. IgM autoantibody might play a protective role against lupus-glomerulonephritis.
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