Hyper IgM syndrome and complement Clq deficiency in an individual with systemic lupus erythematosus-like disease
Tsuge, I.; Kondo, Y.; Nakajima, Y.; Nakagawa, N.; Imai, K.; Nonoyama, S.; Oshima, K.; Ohara, O.; Hatanaka, M.; Kitano, E.; Kitamura, H.; Urisu, A.
Clinical and Experimental Rheumatology 28(4): 558-560
2010
ISSN/ISBN: 0392-856X PMID: 20810037 Document Number: 644543
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.