title: Family study of natural killer cell activity in C1q-deficient patients with systemic lupus erythematosus-like syndrome: association between impaired natural killer cell function and C1q deficiency.
abstract: Impaired natural killer (NK) cell activity has been found in patients with systemic lupus erythematosus (SLE)-like syndrome. The mechanism by which NK cell function is impaired in SLE patients is not quite clear. We report here a family study of NK cell activity in C1q-deficient patients with SLE-like syndrome. In both SLE-active and SLE-inactive stages of the disease, NK cell function was significantly impaired when compared with the healthy controls (10.6 +/- 2.3% and 16.9 +/- 4.8% to 34.7 +/- 9.6%, p less than 0.025). On the other hand, differences in NK cell cytotoxicity between SLE-active and SLE-inactive members of the family were not statistically relevant (p less than 0.1). Further, we found no correlation between NK cell activity and clinical or laboratory values, except for a positive correlation between function of NK cells and C1q and CH50 values, respectively (rs = 0.93, 0.01 less than p less than 0.02). To our knowledge, this is the first report on a notable association between impaired NK cell activity and C1q deficiency. The type of inheritance of C1q deficiency in this family is also discussed.
authors.affiliation: Department of Microbiology and Immunology, Medical Faculty, Comenius University, Bratislava, Czechoslovakia.
authors.forename: J
authors.initials: J
authors.lastname: Tóth
authors.forename: Z
authors.initials: Z
authors.lastname: Starsia
authors.forename: M
authors.initials: M
authors.lastname: Buc
authors.forename: J
authors.initials: J
authors.lastname: Stefanovic
mesh_terms: D000328:Adult; D000936:Antigen-Antibody Complex / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D015922:Complement C1q / Q000172:deficiency* / Q000276:immunology; D015941:Complement Hemolytic Activity Assay; D003601:Cytotoxicity Tests, Immunologic; D005260:Female; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology*; D008180:Lupus Erythematosus, Systemic / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008297:Male; D010375:Pedigree; D013577:Syndrome; D014407:Tumor Cells, Cultured
chemical_list: D000936:Antigen-Antibody Complex; D015922:Complement C1q