Pulmonary hemorrhage in cystic fibrosis: a review of current data

Abstract

Pulmonary hemorrhage is a serious complication of cystic fibrosis (CF) with a prevalence up to 9.1%. According to the Registry of Patients with Cystic Fibrosis of the Russian Federation for 2020, the prevalence of pulmonary hemorrhage was 1.5% in the general population, and 6.5% in patients over 18 years of age. Despite the importance of this problem, there is no systematic information on pulmonary hemorrhage in CF.Aim. The purpose of this study was to review current data on the pathogenesis, risk factors, and approaches to the treatment of pulmonary hemorrhage in patients with CF. Information was searched in еLibrary and PubMed databases.Results. During the literature review, we summarized and systematized the collected scientific data on pulmonary hemorrhage in CF. The pathogenesis of pulmonary hemorrhage is chronic inflammation in the bronchial wall accompanied by increased angiogenesis factors that promote neovascularization with development of many tortuous, thin-walled vessels. Exacerbation of bronchial inflammation and mechanical strain contribute to erosion and damage of fragile vessels and cause the bleeding. The main risk factors for pulmonary hemorrhage were colonization with Pseudomonas aeruginosa, Staphylococcus aureus, diabetes mellitus, and FEV1 < 70%. Complex therapy for pulmonary hemorrhage includes the use of antibiotics and antifibrinolytic agents. Some of the published regimens use β-blockers and blood coagulation factor VII. Data on the efficacy of vitamin K are unclear, but it is also used in the treatment of pulmonary hemorrhage. In cases of massive bleeding, temporarily discontinuation of inhalations of hypertonic solution and kinesiotherapy is recommended. In case of recurrent massive bleedings, bronchial artery embolization is performed and lung resection can be done in extreme cases.Conclusion. Hemoptysis in CF is a complex clinical problem that requires a multidisciplinary approach. Currently, important aspects of the pathogenesis of pulmonary hemorrhage remain unclear. Given the increasing life expectancy of CF patients and the age-related risk of pulmonary hemorrhage, it is important to investigate the pathogenetic aspects of this complication for the subsequent development of effective and justified treatment algorithms.