Document Type: Original Article
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Department of Basic Science and Advanced Technologies in Biology, University of Science and Culture, Tehran, Iran
Background: Common variable immunodeficiency (CVID) is a common primary immunodeficiency disease defined by a failure in B cell differentiation and impaired immunoglobulin production. Subsequently CVID patients are remarkably susceptible to recurrent and multiple infections with bacterial, viral or fungal agents. In the present study, we aimed to provide an update report on different infectious complications of patients with CVID in Iran.
Methods: Demographic, clinical and immunologic data as well as history of infections with related microbial pathogens were obtained from records of patients diagnosed with CVID and followed up at Children’s Medical Center. Based on presence of meningitis, osteomyelitis and sepsis, 2 groups of severe infections and non-severe infections were defined for further investigations.
Results: Among the enrolled 301 CVID patients, 15 (5%) had severe and 286 (95%) had non-severe infections. Respiratory followed by gastrointestinal tract problems (83.1 and 71.4%, respectively) were the most common involved organs. Among the infectious complications, lower and upper respiratory tract infection, followed by mucocutaneous and gastrointestinal tract were the most frequent (76.1, 64.8, 21.6 and 19.6%, respectively). Candida followed by Giardia lamblia were the most common detected pathogens, respectively in those with opportunistic infections and infectious diarrhea.
Conclusion: Recurrent infections of various parts of body are the most prevalent manifestation among patients with CVID playing an important role in the morbidity and even mortality in those with prolonged and untreated infections. Recurrent infections initiating early in childhood should be paid attention and trigger further immunological work up for a possible underlying immunodeficiency especially in families with consanguineous marriage and/or a positive family history of primary immunodeficiency.