title: Poor specific antibody response immunodeficiency (dysgammaglobulinemia) predates systemic lupus erythematosus.
abstract: Poor specific antibody response is a well-known primary immunodeficiency that is related to hypogammaglobulinemia or common variable immunodeficiency (CVID). The co-existence of CVID or hypogammaglobulinemia and systemic lupus erythematosus (SLE) has been rarely described. In all reported cases, the diagnosis of SLE antedates CVID. We report a 15-year-old Saudi girl who was diagnosed with poor specific antibody response at age 6 years in the form of poor or no antibody response and dysgammaglobulinemia. She developed SLE with musculoskeletal and hematological manifestations, positive antinuclear antibody and high anti-dsDNA nine years later. She was treated with rituximab with good response.
authors.affiliation: Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
authors.forename: H
authors.initials: H
authors.lastname: Al Hamzi
authors.forename: A
authors.initials: A
authors.lastname: Al Shaikh
authors.forename: R K
authors.initials: RK
authors.lastname: Arnaout
mesh_terms: D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000276:immunology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000917:Antibody Formation / Q000276:immunology*; D004406:Dysgammaglobulinemia / Q000175:diagnosis* / Q000276:immunology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D000069283:Rituximab; D016896:Treatment Outcome
chemical_list: D000974:Antibodies, Antinuclear; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab
keywords: Systemic lupus erythematosus; T cells and B cells; autoimmunity; common variable immune deficiency; poor specific antibody response; rituximab