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Granulomatous disease in common variable immunodeficiency

L J Mechanic1, S Dikman, C Cunningham-Rundles

  • 1Mount Sinai Medical Center, New York, New York, USA.

Annals of Internal Medicine
|October 27, 1997
PubMed
Summary
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Granulomas in common variable immunodeficiency (CVID) patients may indicate T-cell dysfunction. Early diagnosis of antibody deficiency is crucial to prevent severe illness and autoimmune complications in CVID patients with granulomatous disease.

Area of Science:

  • Immunology
  • Pathology

Background:

  • Granulomatous lesions are observed in lymphoid and solid organs of some patients with common variable immunodeficiency (CVID).
  • The clinical and immunological characteristics of CVID patients with granulomas require further investigation.

Purpose of the Study:

  • To investigate the clinical and immunological profiles of patients diagnosed with common variable immunodeficiency (CVID) who present with granulomas.

Main Methods:

  • A case series study was conducted at a large tertiary care medical center.
  • Seventeen hypogammaglobulinemic patients with CVID and noncaseating granulomas on biopsy were included.
  • Lymphocyte function tests were performed to assess immune status.

Main Results:

  • Eight of 17 patients had granulomas diagnosed before their hypogammaglobulinemia was identified.

Related Experiment Videos

  • Sixteen patients exhibited deficient T-cell proliferation in response to mitogens.
  • Despite receiving intravenous immunoglobulin therapy, 14 patients experienced significant illness, including frequent autoimmune diseases.

    • Conclusions:

    • Dysregulated T-cell function or macrophage activation may contribute to granuloma formation and increased morbidity in CVID patients.
    • Delayed diagnosis of antibody deficiency could exacerbate illness severity in these patients.