Familial selective IgA deficiency with circulating anti-IgA antibodies: A distinct group of patients?

• Published in: Clinical immunology and immunopathology Vol. 58; no. 1; pp. 92 - 101
• Main Authors: de Laat, P.C.J., Weemes, C.M.R., Bakkeren, J.A.J.M., van den Brandt, F.C.A., van Lith, T.G.P.M., de Graaf, R., van Munster, P.J.J., Stoelinga, G.B.A.
• Format: Journal Article
• Language: English
• Published: San Diego, CA Elsevier Inc 1991; New York, NY Academic Press; Boston
• Subjects: Adult; Antibodies, Anti-Idiotypic - analysis; Antibody Formation; Biological and medical sciences; Child; Dysgammaglobulinemia - blood; Dysgammaglobulinemia - genetics; Dysgammaglobulinemia - immunology; Family Health; Female; Humans; IgA Deficiency; Immunodeficiencies. Immunoglobulinopathies; Immunoglobulin A - analysis; Immunoglobulin A - immunology; Immunoglobulinopathies; Immunopathology; Medical sciences; Human; Cell culture; Immunopathology; IgA; Immunoglobulins; Antibody; Family study; Placental transfer; B-Lymphocyte; Immune deficiency; Mechanism; Specificity; Immunoglobulinopathy; Mother; Suppressive cell; T»-Lymphocyte; Child
• ISSN: 0090-1229; 1090-2341
• DOI: 10.1016/0090-1229(91)90151-Y

Two families were investigated in which the mothers had selective IgA deficiency and circulating class-specific anti-IgA antibodies. Both gave birth to two children who were found to be IgA deficient. Three of these children developed anti-IgA antibodies before puberty. In vitro immunoglobulin production studies performed in the children of both families revealed an IgA B cell defect combined with IgA-specific excessive T suppressor function in all four. The mechanisms by which transplacental passage of maternal anti-IgA antibodies could have interfered with the developing IgA system in the offspring are discussed.