Defining Kawasaki Disease and Pediatric Multi-Inflammatory Syndrome During SARS-CoV-2 Epidemic in Italy: Results From A National, Multicenter Survey.

Abstract

Background: There is mounting evidence on the existence of a Pediatric Multi-inflammatory Syndrome related to SARS-CoV-2 (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities.Methods: The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groupsResults: One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis and 37,8% with hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated Ferritin and Troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p=0.04 and 71,9% vs 43,4%; p=0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p<0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%; p<0.0001). Short-term follow data showed minor complications. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data.Conclusion: Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.

Hemolytic anemia following intravenous immunoglobulins in children with PIMS-TS: Two case reports

“…reported to be higher in PIMS-TS than those in KD and represent the overactivation of the immune system (3,4). The diagnosis of PIMS-TS can be confirmed if the following criteria are met: (1) fever > 48 h, (2) elevated inflammatory values such as C-reactive protein, (3) at least two-organ involvement (conjunctivitis, exanthema, gastrointestinal symptoms like diarrhea, abdominal pain, and others), (4) positive SARS-CoV-2 polymerase chain reaction (PCR) or point of care (PoC) antigen tests, positive SARS-CoV-2 antibodies (IgM or IgG), or recent contact with an infected person with SARS-CoV-2, and (5) exclusion of other causes of infection (5). Hyperinflammatory conditions like KD and PIMS-TS are often treated with immunomodulating therapeutic measures like IVIGs.…”