IRCCS G. Gaslini
Rheumatology and Autoinflammatory diseases
Pediatric Rheumatologists and Immunologist. He is Head of the Unit of Rheumatology and Autoinflammatory diseases. His main scientific interests involve the clinical and pathogenic characterization of the monogenic autoinflammatory diseases. He is author of 320 full-papers on international journals (total IF 2760, H-index 78) and of many book chapters. Editor of the book “Familial Mediterranean Fever” (Springer) He received the 1998 and 1999 Pediatric Rheumatology Abstract Awards at the 62nd and 63rd Congress of the American College of Rheumatology and the Kourir Award 13th Congress of Pediatric Rheumatology European Society, Amsterdam, June 2006 2017-2021. President of the International Society of Systemic Autoinflammatory Diseases (ISSAID). 2016-2019: PI of the E-rare project INSAID. From 2008: PI of the “Eurofever” Project (www.printo/eurofever). Past-Chairman of the Working Party for Autoinflammatory diseases of the Pediatric Rheumatology European Society (PRES).

Presenter of 2 Presentations

Mechanistic Overview of Autoinflammatory Diseases

Session Type
Parallel Sessions
Date
Fri, 14.10.2022
Session Time
10:30 - 12:00
Room
Plenary Hall
Lecture Time
10:32 - 10:52

AN UPDATE OF THE INTERNATIONAL REGISTRY ON COVID-19 RELATED HYPERINFLAMMATION IN CHILDREN AND YOUNG ADULTS (HYPERPED-COVID)

Session Type
Oral Communications
Date
Thu, 13.10.2022
Session Time
17:35 - 18:35
Room
Session Hall 02
Lecture Time
18:03 - 18:11

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.

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