The course of cystic fibrosis in adult patients with Burkholderia cepacia complex infection

Cystic fibrosis (CF) is a multi-organ disease, but its course and prognosis in most cases are determined by the pathology of the bronchopulmonary system. One of the most harmful pathogens for patients with CF is Burkholderia cepacia complex (Bcc). These are gram-negative bacteria characterized by high transmissibility and pathogenicity, as well as natural resistance to a wide range of antimicrobial drugs and rapid acquisition of resistance to new antibiotics. This seriously limits the therapeutic possibilities of eradication and treatment of Bcc, progressively reduces the lung function, and significantly limits the life expectancy of patients. A “cepacia syndrome” has also been described for Bcc – a rapidly increasing respiratory failure due to necrotizing pneumonia. Russia belongs to the countries with a relatively high Bcc prevalence of 5.5 %. The aim of the review was to analyze the literature data on the prevalence of Bcc in adult patients with CF in the Russian Federation, the impact of chronic Bcc infection on the course of CF and survival, including after lung transplantation, and the possibility of its eradication and treatment. Burkholderia cenocepacia ST709, an epidemic species that causes a typical hospital infection, the source of which is patients with CF, is more common in Russian patients. No effective schemes for the eradication and treatment of Bcc have been developed. Targeted therapy with ivacaftor + thesacaftor + elecsacaftor and ivacaftor modulator reduce the degree of Bcc contamination in sputum, but do not lead to its eradication. Lung transplantation in patients with Bcc proceeds with a large number of complications and may require long-term rehabilitation in the post-transplant period. Infection with hospital-acquired species of Bcc is associated with a more severe course of the disease and low survival, and infection with less epidemic species of Bcc probably determines the best prognosis for patients with CF. The role of infection of patients with “wild” species of Bcc has not been determined and requires further study. Conclusion. Prevention of cross-infection in patients with Всс remains a pressing issue.