Abstract
Viral infections are known to lead to serious respiratory complications in cystic fibrosis (CF) patients. Hypothesizing that CF patients were a population at high risk for severe respiratory complications from SARS-CoV-2 infection, we conducted a national study to describe the clinical expression of COVID-19 in French CF patients. This prospective observational study involves all 47 French CF centers caring for approximately 7500 CF patients. Between 1 March and 30 June 2020, 31 patients were diagnosed with COVID-19: 19 had positive SARS-CoV-2 RT-PCR in nasopharyngeal swabs; 1 had negative RT-PCR but typical COVID-19 signs on a CT scan; and 11 had positive SARS-CoV-2 serology. Fifteen were males, median (range) age was 31 (9–60) years, and 12 patients were living with a lung transplant. The majority of the patients had CF-related diabetes (n = 19, 61.3%), and a mild lung disease (n = 19, 65%, with percent-predicted forced expiratory volume in 1 s (ppFEV1) > 70). Three (10%) patients remained asymptomatic. For the 28 (90%) patients who displayed symptoms, most common symptoms at admission were fever (n = 22, 78.6%), fatigue (n = 14, 50%), and increased cough (n = 14, 50%). Nineteen were hospitalized (including 11 out of the 12 post-lung transplant patients), seven required oxygen therapy, and four (3 post-lung transplant patients) were admitted to an Intensive Care Unit (ICU). Ten developed complications (including acute respiratory distress syndrome in two post-lung transplant patients), but all recovered and were discharged home without noticeable short-term sequelae. Overall, French CF patients were rarely diagnosed with COVID-19. Further research should establish whether they were not infected or remained asymptomatic upon infection. In diagnosed cases, the short-term evolution was favorable with rare acute respiratory distress syndrome and no death. Post-lung transplant patients had more severe outcomes and should be monitored more closely.
Highlights
Cystic fibrosis (CF) is the most common severe autosomal recessive genetic disease in Caucasians, affecting around 7500 patients in France [1]
Between 1 March and June 2020, patients were diagnosed with COVID-19: 19 had positive SARS-CoV-2 reverse transcription (RT)-PCR in nasopharyngeal swabs; 1 had negative RT-PCR but typical COVID-19 aspects on a CT scan; and 11 had positive SARS-CoV-2 serology
In this national multicenter study, we describe the clinical expression of COVID-19 in French cystic fibrosis (CF) patients over the first few months of the pandemic
Summary
Cystic fibrosis (CF) is the most common severe autosomal recessive genetic disease in Caucasians, affecting around 7500 patients in France [1]. CF is caused by variants in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) [2]. CFTR is an epithelial cell anion channel that plays a major role in the transport of chloride, as well as other anions including bicarbonates (HCO3−), thiocyanate (SCN−), iodide (I−), and glutathione. Lung disease remains the major cause of morbidity and mortality in CF, with a progressive decline of lung function due to a vicious cycle of airway infections and inflammation [5,6]. Among the pathogens infecting the airways in CF patients, viruses are known to contribute to the deterioration of respiratory function, and patients with CF are among those at risk of serious respiratory complications from infection with influenza A virus [7]
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