Presenter of 2 Presentations
Mechanistic Overview of Autoinflammatory Diseases
AN UPDATE OF THE INTERNATIONAL REGISTRY ON COVID-19 RELATED HYPERINFLAMMATION IN CHILDREN AND YOUNG ADULTS (HYPERPED-COVID)
Abstract
Background and Aims
The Multisystem Inflammatory Syndrome in Children (MIS-C), is a serious inflammatory condition characterized by a systemic inflammation with multiorgan failure, that can occur in children and young adults after COVID-19 infection.
Ain of the study is to create an International multicenter collection of patients with MIS-C involving the main pediatric networks committed in the care of patients with hyperinflammatory conditions.
Methods
a steering committee constituted by representatives of ERN-RITA, PRES, ESID and ISSAID and with the coordination of PRINTO developed a shared form to collect clinical manifestations, laboratory features, response to treatment and outcome of patients with MIS-C. The registry is available online on PRINTO and ESID websites (www.printo.it , www.ESID.org).
Results
Currently, more than 1000 patients from 44 centers of 20 countries worldwide have been included in the study; completed data are available for 688 patients. 56 (8%) patients were younger than 2 years, 173 (25%) 2-6 years, 279 (41%) between 6-12 years, while 180 (26%) patients were older than 12 years. 234 (34%) patients required ICU admission; 51 (7.4%) patients presented long term sequaele and 7 (1%) patients died.
Mucocutaneous manifestation were observed in 85.2% of patients, hematological in 84.7%, gastrointestinal in 80.8%, cardiovascular in 52.3%, lymphoid organ in 51.7%, respiratory in 34.6%, musculoskeletal in 31.5%, neurological in 19%, genito-urinary in 10.9%.
576 patients (84%) recieved Ig infusions and 572 (83%) corticosterois; 78 (11%) were treated with biologics.
Conclusions
The first analysis confirms that MISC is a severe inflammatory condition, requiring anti-inflammatory treatment. Even if the mortality rate is low, one third of patients required ICU admission.