Cumulative Incidence and Risk Factors for Severe Coronavirus Disease 2019 in French People With Cystic Fibrosis
Authors: Corvol Harriet , de Miranda Sandra , Dehillotte Clémence , Lemonnier Lydie , Chiron Raphael , Danner-Boucher Isabelle , Hamidfar Rebecca , Houdouin Véronique , Macey Julie , Marguet Christophe , Murris-Espin Marlène , Reynaud Quitterie , Reix Philippe , Reynaud Gaubert Martine , Kemgang Astrid , Burgel Pierre-Régis ,   French Cystic Fibrosis Reference Network Study Group, Abely Michel , Allou Nathalie , ARNOUAT Baptiste , Piccini Carole Bailly , Belleguic Chantal , Bihouee Tiphaine , Billon Yves , Bui Stéphanie , Burgel Pierre-Régis , Camara Boubou , Heraud Marie-Christine , Chiron Raphael , Duet Emmanuelle Coirier , Corvol Harriet , Cosson Laure , Danner-Boucher Isabelle , de Miranda Sandra , Deneuville Eric , Drummond David , Dubus Jean-Christophe , Durieu Isabelle , Epaud Ralph , Gerardin Michèle , Grenet Dominique , Houdouin Véronique , Huet Frédéric , Kanaan Reem , Kessler Romain , Ladaurade Alice , Languepin Jeanne , Laurans Muriel , Rouzic Olivier Le , Leroy Sylvie , Llerena Cathie , Macey Julie , Mankikian Julie , Marguet Christophe , Martin Clémence , Mely Laurent , Mittaine Marie , Murris-Espin Marlène , Perisson Caroline , Prevotat Anne , Ramel Sophie , Rames Cinthia , Reix Philippe , Revillion Marine , Reynaud-Gaubert Martine , Richaud-Thiriez Bénédicte , Scalbert-Dujardin Manuëla , Sermet-Gaudelus Isabelle , Storni Véronique , Tatopoulos Aurélie , Thouvenin Guillaume , Troussier Françoise , Weiss Laurence , Wizla Nathalie 
Published: April 2022

Abstract

Background
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections are closely monitored in people with cystic fibrosis (pwCF), especially severe cases. Previous studies used hospitalization rates as proxy for severity.

Methods
We evaluated data from coronavirus disease 2019 (COVID-19) cases diagnosed in French pwCF over the first pandemic year. Objective criteria were applied for defining severity (eg, respiratory failure and/or death). Data were compared to all French pwCF using the National Registry.

Results
As of 30 April 2021, 223 pwCF were diagnosed with COVID-19, with higher risks in adults (odds ratio [OR], 2.52 [95% confidence interval {CI}, 1.82−3.48]) and transplant recipients (OR, 2.68 [95% CI, 1.98–3.63]). Sixty (26.9%) patients were hospitalized, with increased risk in transplant recipients (OR, 4.74 [95% CI, 2.49–9.02]). In 34 (15%) cases, COVID-19 was considered severe; 28 (46.7%) hospitalizations occurred without objective criteria of severity. Severe cases occurred mostly in adult (85.3%) and posttransplant pwCF (61.8%; OR, 6.02 [95% CI, 2.77–13.06]). In nontransplanted pwCF, risk factors for severity included low lung function (median percentage of predicted forced expiratory volume in 1 second, 54.6% vs 75.1%; OR, 1.04 [95% CI, 1.01–1.08]) and CF-related diabetes (OR, 3.26 [95% CI, 1.02–10.4]). While 204 cases fully recovered, 16 were followed for possible sequelae, and 3 posttransplant females died.

Conclusions
Severe COVID-19 occurred infrequently during the first pandemic year in French pwCF. Nontransplanted adults with severe respiratory disease or diabetes and posttransplant individuals were at risk for severe COVID-19. Thus, specific preventive measures should be proposed.