The role of microbiota in pathogenesis and pathomorphosis of cystic fibrosis

Abstract

The review presents current ideas about the lung microbiota and its important role at different stages of the development of cystic fibrosis, a severe hereditary disease. Just 20 years ago, cystic fibrosis was a fatal disease, but now it demonstrates a tendency towards pathomorphosis, since it becomes a controllable pathology due to basic and targeted pathogenetic therapy. Dysbiosis of the microbial community directly involved in the cystic fibrosis vicious cycle plays a leading role in clinical manifestations of the disease, primarily in the pathology of the respiratory system. New molecular sequencing methods have made it possible to decipher the complex dynamic picture of the respiratory tract microbiome in patients of different age categories. This allowed creating models that most accurately describe the stages of microbiota development depending on the stage of the disease.

Studying pathogenic microbial communities, correlations of their composition with the clinical status of patients, and effects of modulators of the function of the transmembrane regulator of cystic fibrosis open new opportunities in the search for specific biomarkers of the disease to provide an objective prognosis assessment for each patient. Despite its monogenic hereditary nature, cystic fibrosis is a multifactorial disease, in which the interaction of the patient's genotype with the microbiome determines the spectrum of clinical manifestations and the effectiveness of basic and targeted therapy.