Hyper IgM syndrome and complement Clq deficiency in an individual with systemic lupus erythematosus-like disease.
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.
Tsuge
I
I
Department of Paediatrics, Fujita Health University, Aichi, Japan. itsuge@fujita-hu.ac.jp
Kondo
Y
Y
Nakajima
Y
Y
Nakagawa
N
N
Imai
K
K
Nonoyama
S
S
Oshima
K
K
Ohara
O
O
Hatanaka
M
M
Kitano
E
E
Kitamura
H
H
Urisu
A
A
D002648:Child; D015922:Complement C1q / Q000172:deficiency*; D005260:Female; D006801:Humans; D053306:Hyper-IgM Immunodeficiency Syndrome / Q000150:complications* / Q000175:diagnosis; D007075:Immunoglobulin M / Q000502:physiology; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000209:etiology*; D008181:Lupus Nephritis / Q000503:physiopathology / Q000517:prevention & control
D007075:Immunoglobulin M; D015922:Complement C1q