title: Hyper IgM syndrome and complement Clq deficiency in an individual with systemic lupus erythematosus-like disease.
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.
authors.affiliation: Department of Paediatrics, Fujita Health University, Aichi, Japan. itsuge@fujita-hu.ac.jp
authors.forename: I
authors.initials: I
authors.lastname: Tsuge
authors.forename: Y
authors.initials: Y
authors.lastname: Kondo
authors.forename: Y
authors.initials: Y
authors.lastname: Nakajima
authors.forename: N
authors.initials: N
authors.lastname: Nakagawa
authors.forename: K
authors.initials: K
authors.lastname: Imai
authors.forename: S
authors.initials: S
authors.lastname: Nonoyama
authors.forename: K
authors.initials: K
authors.lastname: Oshima
authors.forename: O
authors.initials: O
authors.lastname: Ohara
authors.forename: M
authors.initials: M
authors.lastname: Hatanaka
authors.forename: E
authors.initials: E
authors.lastname: Kitano
authors.forename: H
authors.initials: H
authors.lastname: Kitamura
authors.forename: A
authors.initials: A
authors.lastname: Urisu
mesh_terms: 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
chemical_list: D007075:Immunoglobulin M; D015922:Complement C1q