ABSTRACT
Background: Pediatric Infammatory Multisystemic Syndrome associated to SARS-CoV2 (PIMS) happens 4 to 6 weeks after SARS-CoV2 infection1-2. Its early diagnostic recognition as well as its early management is important to avoid cardiac complications related to this pathology. Objectives: To highlight a frequent symptom in PIMS and improve its therapeutic care. Methods: The JIR Cohort database, an international registry collecting data on patients with pediatric infammatory diseases, was consulted to include patients between 03/15/20 and 12/31/2021. Results: Of the 140 patients in whom a diagnosis of PIMS was retained, we present a series of 38 patients (27%) who presented at diagnosis or during evolution, febrile torticollis or painful cervical involvement. These patients were on average 8.2 years old (0.6-15.2). The proportion of boys was 14 out of 38 (37%). Twenty-four patients out of 33 (73%) were hospitalized in intensive care. Ten patients out of 38 (26%) underwent cervical imaging, 5 (50%) had abnormalities such as collection or infltration of the soft tissues. At the therapeutic level, 27/38 patients (71%) received corticosteroid therapy, 33/38 (87%) immunoglobulins, and 26/38 (68%) antibiotic therapy. Conclusion: PIMS is a pathology with signifcant clinical heterogeneity and severe consequences in case of delay in therapeutic management. In this epidemic context, it is important to consider PIMS in any patient with febrile torticollis, especially if he does not respond to antibiotics.
ABSTRACT
Background: Paediatric infammatory multisystem Syndrome (PIMS) is a new systemic infammatory disease linked to SARS-CoV2 that affects children. It was frst reported in may 2020 [1-2]. Objectives: The objectives of this study were to describe patients with PIMS through the international JIR cohort registry and to compare the different profiles and treatments of these patients over the different waves. Methods: Study patients with international PIMS criteria were included from March 2020 to June 2021. Patients were identifed in the JIR cohort, an international registry collecting demographic, clinical and paraclinical data on patients with pediatric infammatory diseases. Two groups were distinguished: from March 2020 to July 2020 for patients in the frst wave, from July 2020 to June 2021 for patients in the 2nd and 3rd waves. These two groups were compared using a Fischer test for categorical data and a Mann-Whitney test for quantitative data Results: 136 patients meeting the PIMS criteria were included (64 patients in the 1st wave, 72 patients after). Patients had less frequent myocarditis (51 patients in wave 1 vs. 36 patients after, p=0,0003) and respiratory distress (34 patients vs 10 patients, p<0,0001). Corticosteroids were used more frequently in the second wave (32 patients in wave 1 vs. 67 patients after July 2020, p<0,0001). Intravenous immunoglobulins were used as much over the waves (58 patients in wave 1 vs 68 patients after, p=0.5). Antibiotics were less used since the second wave (53 patients received antibiotics before July 2020 vs 11 after, p<0,0001). The duration of hospitalization decreased sig-nifcantly (p<0,0001) with a median duration of 9 days during the frst wave (interquartile range, 7-12) and 7 days (interquartile range, 5-10) after the frst wave. Conclusion: There was a decrease in the number of complications of PIMS, particularly cardiac and respiratory complications, and a decrease in the length of hospitalization over time. The treatment of PIMS has also evolved, with a clear increase in the use of corticosteroids and a decrease in the use of antibiotics.
ABSTRACT
Introduction: At the end of April 2020, European clinicians warned the Public Health Agencies about an abnormal increase of Kawasakilike diseases and myocarditis requiring critical care support in the context of the ongoing COVID-19 epidemic in children. American clinicians also reported a large outbreak of severe inflammation in children following COVID-19 infection, a condition that is now named Pediatric Inflammatory Multisystemic Syndrome (PIMS) or Multisystem Inflammatory Syndrome in children (MIS-C). Objectives: As MIS-C combines clinical features of Kawasaki disease (KD) and Toxic Shock Syndrome (TSS), we aimed to compare the immunological profile of pediatric patients with these different conditions. Methods: We analysed blood cytokine expression, and the T cell repertoire and phenotype in 36 MIS-C cases, which were compared to 16 KD, 58 TSS, and 42 COVID-19 cases. Results: We observed an increase of serum inflammatory cytokines (IL-6, IL-10, IL-18, TNF-a, IFNg, CD25s, MCP1, IL-1RA) in MIS-C, TSS and KD, contrasting with low expression of HLA-DR in monocytes. We detected a specific expansion of activated T cells expressing the Vβ21.3 T cell receptor β chain variable region in both CD4 and CD8 subsets in 75% of MIS-C patients and not in any patient with TSS, KD, or acute COVID-19;this correlated with the cytokine storm detected. The T cell repertoire returned to baseline within weeks after MIS-C resolution. Vβ21.3+ T cells from MIS-C patients expressed high levels of HLA-DR, CD38 and CX3CR1 but had weak responses to SARS-CoV- 2 peptides in vitro. Consistently, the T cell expansion was not associated with specific classical HLA alleles. Conclusion: Thus, our data suggested that MIS-C is characterized by a polyclonal Vβ21.3 T cell expansion not directed against SARS-CoV-2 antigenic peptides, which is not seen in KD, TSS and acute COVID-19.
ABSTRACT
Background: Cardiac injury and myocarditis have been described in adults with coronavirus disease 2019 (COVID-19). Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children is typically minimally symptomatic. We report a series of febrile pediatric patients with acute heart failure potentially associated with SARS-CoV-2 infection and the multisystem inflammatory syndrome in children as defined by the US Centers for Disease Control and Prevention. Methods: Over a 2-month period, contemporary with the SARS-CoV-2 pandemic in France and Switzerland, we retrospectively collected clinical, biological, therapeutic, and early outcomes data in children who were admitted to pediatric intensive care units in 14 centers for cardiogenic shock, left ventricular dysfunction, and severe inflammatory state. Results: Thirty-five children were identified and included in the study. Median age at admission was 10 years (range, 2-16 years). Comorbidities were present in 28%, including asthma and overweight. Gastrointestinal symptoms were prominent. Left ventricular ejection fraction was <30% in one-third;80% required inotropic support with 28% treated with extracorporeal membrane oxygenation. Inflammation markers were suggestive of cytokine storm (interleukin-6 median, 135 pg/mL) and macrophage activation (D-dimer median, 5284 ng/mL). Mean BNP (B-type natriuretic peptide) was elevated (5743 pg/mL). Thirty-one of 35 patients (88%) tested positive for SARS-CoV-2 infection by polymerase chain reaction of nasopharyngeal swab or serology. All patients received intravenous immunoglobulin, with adjunctive steroid therapy used in one-third. Left ventricular function was restored in the 25 of 35 of those discharged from the intensive care unit. No patient died, and all patients treated with extracorporeal membrane oxygenation were successfully weaned. Conclusions: Children may experience an acute cardiac decompensation caused by severe inflammatory state after SARS-CoV-2 infection (multisystem inflammatory syndrome in children). Treatment with immunoglobulin appears to be associated with recovery of left ventricular systolic function.