Autoimmunity in a cohort of 471 patients with primary antibody deficiencies

Expert Rev Clin Immunol. 2017 Nov;13(11):1099-1106. doi: 10.1080/1744666X.2017.1384312. Epub 2017 Oct 11.


Objectives: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD).

Methods: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications.

Results: Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2.5%). History of autoimmunity was recorded in 125 patients during the course of the disease (26.5%). The frequency of autoimmunity in common variable immune deficiency (32.0%) was higher than other forms of PADs. The most common autoimmune manifestations were reported to be autoimmune gastrointestinal disease and autoimmune cytopenias. Among patients with autoimmunity, 87 patients (69.6%) had a history of one autoimmune disorder, while 38 patients (30.4%) had a history of multiple autoimmunities. The immune thrombocytopenic purpura and autoimmune hemolytic anemia were the most two concomitant autoimmune disorders in 16 (42.1%) of 38 patients with multiple autoimmunities. Comparing the frequency of Tregs in PAD patients with autoimmunity showed that, patients with multiple autoimmunities had lower Tregs than those with single autoimmunity (p = 0.017).

Conclusion: It is important that non-immunologist physicians be alert of the associated autoimmunity with PADs in order to reduce the diagnostic delay and establish timely immunoglobulin replacement therapy in these patients.

Keywords: Primary antibody deficiencies; autoimmune hemolytic anemia; autoimmunity; immune thrombocytopenic purpura.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Hemolytic, Autoimmune / epidemiology*
  • Anemia, Hemolytic, Autoimmune / immunology
  • Autoantibodies / metabolism
  • Autoimmunity
  • Child
  • Cohort Studies
  • Common Variable Immunodeficiency / epidemiology*
  • Common Variable Immunodeficiency / immunology
  • Delayed Diagnosis
  • Female
  • Gastrointestinal Diseases / epidemiology*
  • Gastrointestinal Diseases / immunology
  • Humans
  • Immunologic Deficiency Syndromes / epidemiology*
  • Immunologic Deficiency Syndromes / immunology
  • Male
  • Purpura, Thrombocytopenic, Idiopathic / epidemiology*
  • Purpura, Thrombocytopenic, Idiopathic / immunology
  • Retrospective Studies
  • T-Lymphocytes, Regulatory / immunology*
  • Young Adult


  • Autoantibodies