DISSEMINATED BCG INFECTION IN TUNISIA: CLINICAL AND AETIOLOGICAL FEATURES

Disseminated BCG infection (BCGitis) is a rare complication of antituberculous vaccination and usually affects children presenting an immune deficiency. We report the Tunisian experience of about 12 cases of disseminated BCGitis. Methods We retrospectively studied 12 cases of disseminated BCGitis enrolled into the paediatric departments of Tunisia during a 15 year period (1990–2004). Results There were 9 boys and 3 girls belonging to 11 unrelated Tunisian families. Parental consanguinity was noted in the half of cases and deaths in the low age in 2 cases. After BCG vaccine was received in all cases at birth, the first clinical symptoms appeared at age 3 months on average (extreme:16 days to 5 months) whereas age of diagnosis was 9 months on average (extreme:3 to 28 months). Immunogenetic studies identified immune deficiency in 10 cases: IL12p40 deficiency in 5 cases, IL12 receptor β1 deficiency in 2 cases, severe combined immunodeficiency in 2 cases and chronic granulomatous disease in 1 case. Immunological investigation remained negative in 2 cases. All patients have received antituberculous treatment and 2 among them interferon gamma. Evolution was favourable in only the half of cases. The 6 other patients died: 2 patients at an evolved stage of the disease and 4 by severe infection. Conclusions BCG vaccination, which is compulsory at birth in Tunisia, had probably favoured the emergence of disseminated BCGitis occurring in genetically immunodeficient children. Disseminated BCGitis cases were clinically and genetically heterogeneous. However, genetic defects in the IL12/IFN pathway found in 7 cases were the most frequent aetiology of this disorder.