The effect of nintedanib on lung functions and survival in idiopathic pulmonary fibrosis: real-life analysis of the Czech EMPIRE registry.

INTRODUCTION: The antifibrotic drug nintedanib is used for the treatment of idiopathic pulmonary fibrosis (IPF). We analysed the effect of nintedanib on antifibrotic treatment outcome in real-world cohorts of Czech EMPIRE registry. PATIENTS/METHODS: Data of 611 Czech IPF subjects, 430 (70%) treated with nintedanib (NIN group), 181 (30%) with no-antifibrotic treatment (NAF group) were analysed. The influence of nintedanib on overall survival (OS), pulmonary function parameters as forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO), as well as GAP score (gender, age, physiology) and and CPI (composite physiological index) were investigated. RESULTS: During 2 year follow-up we observed that nintedanib treated patients had longer OS, compared to those treated with no-antifibrotic drugs (p < 0.00001). Nintedanib reduces risk of mortality over no-antifibrotic treatment by 55% (p < 0.001). We have observed no significant difference in the rate of FVC and DLCO decline between the NIN and NAF group. Changes within 24 months from baseline in CPI were not significant between the groups (NAF and NIN). CONCLUSION: Our real-practice study showed the benefit of nintedanib treatment on survival. There were no significant differences between NIN and NAF groups in changes from baseline in FVC %, DLCO % predicted and CPI.

Adherence to the ISHLT Protocol for the Referral of Patients with Idiopathic Pulmonary Fibrosis to the Transplantation Center among of Czech Centers for Interstitial Lung Diseases.

There are limited data on referral rates and the number of patients with idiopathic pulmonary fibrosis (IPF) who are eligible for lung transplantation. The aim of the present study was to assess adherence to the consensus of the International Society for Heart and Lung Transplantation (ISHLT) for the referral of patients with IPF among Czech interstitial lung disease (ILD) centers. Czech patients who were diagnosed with IPF between 1999 and 2021 (n = 1584) and who were less than 65 years old at the time of diagnosis were retrospectively selected from the Czech Republic of the European Multipartner Idiopathic Pulmonary Fibrosis Registry (EMPIRE). Nonsmokers and ex-smokers with a body mass index (BMI) of <32 kg/m2 (n = 404) were included for further analyses. Patients with a history of cancer <5 years from the time of IPF diagnosis, patients with alcohol abuse, and patients with an accumulation of vascular comorbidities were excluded. The trajectory of individual patients was verified at the relevant ILD center. From the database of transplant patients (1999-12/2021, n = 541), all patients who underwent transplantation for pulmonary fibrosis (n = 186) were selected, and the diagnosis of IPF was subsequently verified from the patient's medical records (n = 67). A total of 304 IPF patients were eligible for lung transplantation. Ninety-six patients were referred to the transplant center, 50% (n = 49) of whom were referred for lung transplantation. Thirty percent of potentially eligible patients not referred to the transplant center were considered to have too many comorbidities by the reporting physician, 19% of IPF patients denied lung transplantation, and 17% were not referred due to age. Among Czech patients with IPF, there may be a larger pool of potential lung transplant candidates than has been reported to the transplant center to date.

Cardiac sequelae after COVID-19: Results of a 1-year follow-up study with echocardiography and biomarkers.

Objective: To evaluate the need for cardiac monitoring in unselected patients recovered from COVID-19 and to estimate the risk of heart complications after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Materials and methods: During March 2020 and January 2021, 106 patients who had recovered from SARS-CoV-2 (alpha and beta variants) were enrolled in prospective observational cohort study CoSuBr (Covid Survivals in Brno). The diagnosis was based on a reverse transcription-polymerase chain reaction swab test of the upper respiratory tract. Demographic parameters, patient history, clinical evaluation, cardiac biomarkers, ECG and echocardiography were recorded during three visits (Visit 1 at least 6 weeks after infection, Visit 2 three months later, and Visit 3 one year after Visit 1). Results: 58.5% of the study group (n = 106) were female, while the mean age was 46 years (range 18-77 years). The mean time interval between the onset of infection and the follow-up visit was 107 days. One quarter (24.5%) of the patients required hospitalization during the acute phase of the disease; the rest recovered at home. 74% suffered a mild form of the disease, with 4.8, 18.1, and 2.9% suffering moderate, severe, and critical forms, respectively. At the time of enrolment, 64.2% of the patients reported persistent symptoms, while more than half of the whole group (50.9%) mentioned at least one symptom of possible cardiac origin (breathing problems, palpitations, exercise intolerance, fatigue). In the 1-year follow-up after COVID-19 infection, left ventricle ejection fraction showed no significant decrease [median (IQR) change was -1.0 (-6.0; 4.0)%, p = 0.150], and there were no changes of troponin (mean change -0.1 ± 1.72 ng/L; p = 0.380) or NT-proBNP [median (IQR) change 2.0 (-20.0; 29.0) pg/mL; p = 0.315]. There was a mild decrease in right ventricle end diastolic diameter (-mean change 2.3 ± 5.61 mm, p < 0.001), while no right ventricle dysfunction was detected. There was very mild progress in left ventricle diastolic diameter [median (IQR) change 1.0 (-1.0; 4.0) mm; p = 0.001] between V1 and V3, mild enlargement of the left atrium (mean change 1.2 ± 4.17 mm; p = 0.021) and a non-significant trend to impairment of left ventricle diastolic dysfunction. There was a mild change in pulmonary artery systolic pressure [median (IQR) change 3.0 (-2.0; 8.0) mmHg; p = 0.038]. Conclusion: Despite a lot of information regarding cardiac impairment due to SARS-CoV2, our study does not suggest an increased risk for developing clinically significant heart changes during the 1-year follow-up. Based on our results, routine echocardiography and biomarkers collection is currently not recommended after COVID-19 recovery.