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.

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020

“…In the ECFSPR geographic area, the overall incidence of SARS-CoV-2 in pwCF was not significantly different to the incidence of that of the corresponding general population (2.70 versus 3.10/1000). SARS-CoV-2 was lower in pwCF versus the general population in studies from a global group (0.7/1000 versus 1.5/1000 up to mid-April 2020) [7] , Spain (3.2/1000 versus 4.9/1000 up to 16 May 20) [19] , France (4.1/1000 pwCF up to 30 June 20) [5] , Italy (15.8/1000 versus 29.1/1000 up to November 2020) [20] and Verona, Italy (1.9/1000 versus 4/1000 up to 23 July 20) [21] .…”

“…Upon SARS-CoV-2 infection, lung-transplanted pwCF were more frequently symptomatic, were admitted to hospital 1.7-fold more frequently and required intensive care 8-fold more frequently than pwCF without lung transplant. Previous studies also found that solid organ transplant recipients require more treatment and medical care upon SARS-CoV-2 infection, whether transplant was for CF [ 5 , 7 ] or other reasons [ 30 , 31 ].…”

“…Therefore, the SARS-CoV-2 pandemic caused considerable anxiety in the CF community [4] . Early case series of pwCF in various regions who tested positive for SARS-CoV-2 suggest that pwCF have better outcomes than expected [5] , [6] , [7] , [8] .…”

SARS-CoV-2 infections in people with PCD: neither frequent, nor particularly severe

“…Among the 31, 61% had been hospitalized and 13% (4/31) were in the ICU. [12]. For PCD, we found that most people who tested positive for SARS-COV-2 had mild symptoms, only 1 (8%) was hospitalised and none was in the ICU.…”

“…Results overall are comparable to what has been found for cystic fibrosis (CF). A French study of 7500 CF patients from 47 clinics found that 31 had tested positive for SARS-CoV-2 by June 2020, 0.4% of the study population [12]. Among the 31, 61% had been hospitalized and 13% (4/31) were in the ICU.…”