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Original Article
 

Autoimmune Phenotype in Patients With Common Variable Immunodeficiency
 

H Abolhassani,1 D Amirkashani,1 N Parvaneh,1 P Mohammadinejad,1
B Gharib,1 S Shahinpour,1 A Hirbod-Mobarakeh,1 M Mirghorbani,1
M Movahedi,2 M Gharagozlou,2 N Rezaei,1 A Aghamohammadi1

1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
2Department of Allergy and Clinical Immunology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran

J Investig Allergol Clin Immunol 2013; Vol. 23(5): 323-329
 

 Abstract


Background and objective: Autoimmune disorders occur with a higher incidence in common variable immunodeficiency (CVID) patients than in the general population. To describe the clinical features of the autoimmune phenotype in patients with CVID.

Methods: The hospital records of all diagnosed CVID patients referred to the Children’s Medical Center Hospital in Tehran, Iran between 2000 and 2010 were reviewed. Patients were also classified according to the presence or absence of autoimmune disease.

Results: Of 52 patients studied, 26.9% (n=14) had shown at least 1 autoimmune manifestation during the study period. Autoimmune cytopenias and juvenile rheumatoid arthritis were the most common form of autoimmunity in our series. Autoimmunity was significantly associated with polyclonal lymphocytic infiltrative disorders (P=.017), increased serum Immunoglobulin (Ig) M levels (P<.001), decreased IgE values (P=.04) and diminished switched memory B-cell count (P<.001).

Conclusions: Because autoimmunity is one of the first manifestations in CVID, humoral immune system tests should be considered in autoimmune patients with a history of recurrent infection. The presence of polyclonal lymphocytic infiltrative disorders and decreased switched memory B-cells may predispose CVID patients to autoimmunity.

Key words: Autoimmune disorders. Common variable immunodeficiency. Clinical phenotypes.