Risk factors for severe COVID-19 in people with cystic fibrosis: A systematic review

Abstract

BackgroundPeople with cystic fibrosis (CF) are considered a clinically fragile population with an intrinsic higher risk of developing severe COVID-19, though a certain variability in terms of outcomes and hospitalization has been noticed.AimTo highlight the main risk factors for severe COVID-19 in patients with CF.MethodsA systematic review of the current literature was conducted through PubMed and EMBASE databases. English-written articles reporting clinical data on CF subjects with SARS-CoV2 infection were included and analyzed. Selected reports were evaluated for adherence to STROBE recommendations.ResultsAfter the selection phase, 9 observational studies were included, 5 of which reported data from CF Registry Global Harmonization Group. The hospitalization rate ranged from 18.2 to 58.1%. The main risk factors for severe outcome were as follows: FEV1 < 70%p, CF-related diabetes, age > 40 years, pancreatic insufficiency, underweight, previous transplant, azithromycin use. Use of dornase alfa was associated with decreased risk for severe disease, while there was insufficient evidence to establish the role of inhaled steroids or CFTR modulators. No solid data regarding specific SARS-CoV-2 therapies in patients with CF emerged.ConclusionMost people with CF experience a mild course of SARS-CoV-2 infection, nevertheless subgroups with higher risk of severe outcome emerged. Maintenance therapies for CF overall did not show a clear preventive effect against severe outcomes, although dornase alfa seems to give some protection. Due to the current lack of data on specific COVID-19 therapies and immunization in patients with CF, further studies are needed to establish their impact in this population.

Impact of SARS-CoV-2 infection and COVID-19 on patients with inborn errors of immunity

“…Here, it was found that many cystic fibrosis patients had mild disease and common risk factors such as diabetes and previous solid organ transplantation, but subgroups of patients exhibited increased hospitalization rates and younger age at presentation relative to the general population. 117

Fig 1 Features of cohorts of patients with IEI and SARS-CoV-2 infection. (A) Age of patients with the indicated IEI.

…”

A year in review (2022): Modulators and COVID19, the story goes on…

“…A systematic review of the risk factors for severe COVID-19 disease in pwCF [1] identified the main risk factors for hospitalization included percent predicted forced expiratory volume in 1 second (ppFEV 1 ) <70, CF-related diabetes (CF-RD), age >40 years, pancreatic insufficiency, lower body mass index (BMI) and previous lung transplant. In the pediatric CF population lung function and BMI Z-score were both found to be significantly lower in hospitalized children, compared to non-hospitalized ones.…”

Long-Term Assessment of Antibody Response to COVID-19 Vaccination in People with Cystic Fibrosis and Solid Organ Transplantation

“…Data from the European Cystic Fibrosis Patient Registry showed that general symptoms were the most common in the context of a COVID-19 infection, including fever, fatigue, myalgia/arthralgia and headache, followed by increased cough and pulmonary exacerbation [ 8 ]. However, transplant status was still considered one of the main risk factors for serious outcomes and these patients were therefore given priority in the vaccination program [ 9 ]. In view of the rapid spread of the infection, vaccines were developed very quickly.…”