PT - JOURNAL ARTICLE AU - Rim Bourguiba AU - Maeva Kyheng AU - Isabelle Koné-Paut AU - Diane Rouzaud AU - Jerome Avouac AU - Mathilde Devaux AU - Nassim Ait Abdallah AU - Bruno Fautrel AU - Nicole Ferreira-Maldent AU - Vincent Langlois AU - Emmanuel Ledoult AU - Hubert Nielly AU - Viviane Queyrel AU - Jérémie Sellam AU - Nathalie Tieulie AU - Pascal Chazerain AU - Philippe Evon AU - Julien Labreuche AU - Léa Savey AU - Veronique Hentgen AU - Gilles Grateau AU - Sophie Georgin-lavialle ED - , TI - COVID-19 infection among patients with autoinflammatory diseases: a study on 117 French patients compared with 1545 from the French RMD COVID-19 cohort: COVIMAI – the French cohort study of SARS-CoV-2 infection in patient with systemic autoinflammatory diseases AID - 10.1136/rmdopen-2021-002063 DP - 2022 May 01 TA - RMD Open PG - e002063 VI - 8 IP - 1 4099 - http://rmdopen.bmj.com/content/8/1/e002063.short 4100 - http://rmdopen.bmj.com/content/8/1/e002063.full SO - RMD Open2022 May 01; 8 AB - Objective There is little known about SARS-CoV-2 infection in patients with systemic autoinflammatory disease (SAID). This study aimed to describe epidemiological features associated with severe disease form and death. Mortality between patients with and without SAID hospitalised for SARS-CoV-2 infection was compared.Methods A national multicentric prospective cohort study was conducted from the French Rheumatic and Musculoskeletal Diseases (RMD) COVID-19 cohort. Patients with SAID were matched with patients with non-SAID on age±7 years, gender and number of comorbidities to consider important confounding factors. Impact of SAID on severity of SARS-CoV-2 infection was analysed using multinomial logistic regression for severity in three classes (mild, moderate and severe with mild status as reference). Fine-Gray regression model for length of hospital stay and binomial logistic regression model for risk of death at 30 days.Results We identified 117 patients with SAID (sex ratio 0.84, 17 children) and compared them with 1545 patients with non-autoinflammatory immune-mediated inflammatory disorders (non-SAID). 67 patients had a monogenic SAID (64 with familial Mediterranean fever). Other SAIDs were Behçet’ disease (n=21), undifferentiated SAID (n=16), adult-onset Still disease (n=9) and systemic-onset juvenile idiopathic arthritis (n=5). Ten adults developed severe form (8.6%). Six patients died. All children had a benign disease. After matching on age±7 years, sex and number of comorbidities, no significant difference between the two groups in length of stay and the severity of infection was noted.Conclusion As identified in the whole French RMD COVID-19 cohort, patients with SAID on corticosteroids and with multiple comorbidities are prone to develop more severe COVID-19 forms.