Case Reports

Hyper IgM syndrome and complement Clq deficiency in an individual with systemic lupus erythematosus-like disease.

I. Tsuge, Y. Kondo, Y. Nakajima, N. Nakagawa, K. Imai, S. Nonoyama, K. Oshima, O. Ohara, M. Hatanaka, E. Kitano, H. Kitamura, A. Urisu

CER904
2010 Vol.28, N°4
PI 0558, PF 0560
Case Reports

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PMID: 20810037 [PubMed]

Received: 12/02/2010
Accepted : 25/06/2010
In Press: 30/08/2010
Published: 30/08/2010

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.