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Intestinal B cell defects in common variable immunodeficiency

E W Herbst1, M Armbruster, J A Rump

  • 1Pathologisches Institut, Universität Freiburg, Germany.

Clinical and Experimental Immunology
|February 1, 1994
PubMed
Summary
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Common variable immunodeficiency (CVID) patients often lack essential B cells in their small intestine, impacting immune function. Intestinal biopsies are crucial for diagnosing mucosal immunodeficiency in CVID.

Area of Science:

  • Immunology
  • Gastroenterology
  • Cell Biology

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low immunoglobulin levels.
  • The small intestine's humoral immune system plays a critical role in mucosal defense.
  • Understanding B cell populations in the CVID gut is essential for diagnosing and managing complications.

Purpose of the Study:

  • To investigate the presence and distribution of immunoglobulin-expressing B cells in the small intestinal mucosa of CVID patients.
  • To correlate B cell findings with clinical manifestations, specifically intestinal infections.
  • To assess the utility of peripheral blood lymphocyte assays versus intestinal biopsies for evaluating mucosal immunity in CVID.

Main Methods:

  • Immunohistology was performed on small intestinal biopsies from 17 CVID patients.

Related Experiment Videos

  • Antibodies specific for IgA1, IgA2, IgM, IgG1-4, J chain, and secretory component (SC) were used.
  • Conventional staining assessed plasma cell proportions; in vitro immunoglobulin synthesis assays were also conducted.

    • Main Results:

    • Significant deficiencies in IgA, IgG2, and IgM-expressing B cells were observed in the lamina propria of CVID patients.
    • J chain-synthesizing cells were present, indicating blocked B cell differentiation.
    • Intestinal IgA defects correlated with infections by Giardia lamblia, Campylobacter jejuni, or Candida albicans.
    • Secretory component expression on enterocytes appeared independent of immunoglobulin levels.
    • Peripheral blood assays did not predict mucosal B cell presence.

    Conclusions:

    • CVID patients exhibit substantial defects in intestinal humoral immunity, with frequent absence of IgA, IgG2, and IgM B cells.
    • Intestinal biopsies are necessary for accurate assessment of mucosal immunodeficiency in CVID, as peripheral blood tests are unreliable.
    • IgA deficiency is linked to increased susceptibility to specific intestinal pathogens in CVID.