First Wave of COVID-19 in French Patients with Cystic Fibrosis

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 March 1st and June 30th 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.

Keywords: COVID-19; Cystic Fibrosis; SARS-CoV-2; acute respiratory distress syndrome; lung transplant.

Figure 1

Distribution of the patients with cystic fibrosis at SARS-CoV-2 infection onset according to age-categories. Among the 31 included patients, a majority were adults (≥18 years) with 14 patients (45%) aged between 30–49 years-old, and 9 (29%) between 18–29 years-old. Only 6 patients (19%) were children.

Figure 2

Distribution of baseline lung function in 31 patients with CF at SARS-CoV-2 infection onset. At SARS-CoV-2 infection onset, among the 31 patients, the majority had preserved lung function, 19 patients (65%) having a percent-predicted forced expiratory volume in one second (ppFEV₁) that was greater than 70.

Figure 3

Distribution of the main symptoms at SARS-CoV-2 infection at onset in the patients with cystic fibrosis. Among the 31 patients, 28 (90%) had symptoms of SARS-CoV-2 infection at onset, whereas 3 (10%) remained asymptomatic.