Panhypogammaglobulinemia in systemic lupus erythematosus: in vitro demonstration of multiple cellular defects. NLM AIDSLINE Important note: Information in this article was accurate in 1983. The state of the art may have changed since the publication date.

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Panhypogammaglobulinemia in systemic lupus erythematosus: in vitro demonstration of multiple cellular defects.

J Allergy Clin Immunol. 1982 Dec;70(6):465-73. Unique Identifier : AIDSLINE MED/83057778
Ashman RF; White RH; Wiesenhutter C; Cantor Y; Lasarow E; Liebling M; Talal N


Abstract: Classically, systemic lupus erythematosus (SLE) is a disease of antibody overproduction, whereas the hallmark of acquired immune deficiency is antibody underproduction. Two patients are presented in whom panhypogammaglobulinemia developed during the course of SLE. In both patients, the levels of the major immunoglobulin (Ig) classes did not fall simultaneously. Anti-DNA antibodies were present, and exacerbations of SLE nephritis occurred in both cases 6 to 8 yr after Ig levels became subnormal. One patient still requires immunosuppressive therapy for renal disease; both patients are experiencing recurrent sinopulmonary bacterial infections. In the pokeweed mitogen--stimulated Ig biosynthesis assay, both patients showed abnormal Ig production due to defective function of three cell types: hyporesponsive B cells, excessive T suppression, and subnormal T help. The latter defect is rare in common variable hypogammaglobulinemia. One patient also showed extreme suppression of Ig production by phagocytic mononuclear cells. Thus, despite the similarity in the histories, the cellular function of these two patients was not identical in vitro.
Keywords: Adult Agammaglobulinemia/COMPLICATIONS/*IMMUNOLOGY Antibody-Producing Cells/IMMUNOLOGY B-Lymphocytes/IMMUNOLOGY Case Report Female Human IgA/BIOSYNTHESIS IgG/BIOSYNTHESIS IgM/BIOSYNTHESIS Immunity, Cellular Lupus Erythematosus, Systemic/COMPLICATIONS/*IMMUNOLOGY Lymphocyte Transformation Macrophages/IMMUNOLOGY Support, U.S. Gov't, P.H.S. T-Lymphocytes, Helper-Inducer/IMMUNOLOGY T-Lymphocytes, Suppressor-Effector/IMMUNOLOGY JOURNAL ARTICLE

KWDadultagammaglobulinemia/complications/KWDimmunologyantibody-producingcells/immunologyb-lymphocytes/immunologycasereportfemalehumaniga/biosynthesisigg/biosynthesisigm/biosynthesisimmunity,cellularlupuserythematosus,systemic/complications/KWDimmunologylymphocytetransformationmacrophages/immunologysupport,uKWDsKWDgov't,pKWDhKWDsKWDt-lymphocytes,helper-inducer/immunologyt-lymphocytes,suppressor-effector/immunologyjournalarticle
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Copyright © 1983 - National Library of Medicine. Reproduced under license with the National Library of Medicine, Bethesda, MD.

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