Disseminated BCG Infection and Primary Immunodeficiencies: A Report from Two Tertiary Centers

Abstract

Background: One of the most serious complications of BCG immunization is disseminated BCG infection, which is suggested to occur in immunized children with underlying primary immunodeficiencies. Objectives: This study aimed to assess the clinical manifestations and underlying primary immunodeficiencies associated with disseminated BCG infection. Methods: The study enrolled 47 patients suspected of disseminated BCG infection referring to Mofid Children Hospital and Masih Daneshvari Hospital for 12 years. The patients’ records were reviewed and patients were classified into three distinctive groups of definitive, probable, or possible disseminated BCG infection. Results: Twenty-five (53.2%) patients were male and twenty-two (46.8%) were female. The mean age at onset of clinical manifestations was 4.83 months. The first presentation of the disease occurred within one year of vaccination in 28 (60%) patients. Clinical manifestations included lymphadenopathies (61%), fever (38%), hepatosplenomegaly (36%), failure to thrive (23%), skin rash (14%), chronic cough (10%), ascites (6%), and clubbing (6%). The confirmed underlying primary immunodeficiency detected in these patients were Mendelian Susceptibility to Mycobacterial Disease (MSMD; 69.56%), Severe Combined Immunodeficiency (SCID; 26%) and Chronic Granulomatous Disease (CGD; 4.3%). Conclusions: Disseminated BCG infection may be a devastating complication and an important preliminary manifestation of underlying primary immunodeficiency. Because of the wide spectrum of mortality and morbidity, as well as the socioeconomic burden on the health system, it is worth to take a careful medical history before BCG immunization particularly in families with a history of consanguineous marriage and death due to unknown etiology.