Autor:innen:
S. Hébert (Erlangen, DE)
M. Schmidt (Erlangen, DE)
G. Wallukat (Berlin, DE)
R. Ponader (Erlangen, DE)
G. Topf (Furth, DE)
D. Rieger (Furth, DE)
J. Klinge (Furth, DE)
J. Vermehren (Nuremberg, DE)
C. Fusch (Nuremberg, DE)
C. Grillhoesl (Nuremberg, DE)
M. Schroth (Nuremberg, DE)
T. Krickau (Erlangen, DE)
I. Toni (Erlangen, DE)
H. Reutter (Erlangen, DE)
P. Morhart (Erlangen, DE)
G. Hanslik (Erlangen, DE)
L. Mulzer (Erlangen, DE)
J. Woelfle (Erlangen, DE)
A. Agaimy (Erlangen, DE)
B. Hohberger (Erlangen, DE)
A. Hoerning (Erlangen, DE)
Background:
Multisystemic inflammatory syndrome in children (MIS-C) is a rare autoimmune reaction that occurs after a latency period following SARS-CoV-2 infection. Its clinical symptoms are similar to the Kawasaki disease including hyperinflammation, gastrointestinal, cardiac, respiratory, and neurological symptoms. Here, we aimed to investigate the incidence, therapeutical procedure and outcome of MIS-C patients in the metropolitan area of Nuremberg-Erlangen, comprising 3,6 million habitants. Further, we report patient with MIS-C with predominating gastrointestinal symptoms associated with SARS-CoV2 mRNA vaccination.
Material and Methods:
Clinical reports were screened for MIS-C in all Children Hospitals in the metropolitan area Nuremberg-Erlangen comprising 3,6 million habitants within the time period 01/2021 -12/2022. Symptoms, therapies, and outcomes are presented.
Results:
We identified 10 patients with MIS-C, at an average of 5,9 weeks after PCR confirmed COVID-19 infection. Mean age was 11. Symptoms comprised fever (n=10), a rash (n=6), bilateral non-purulent conjunctivitis (n=5), and urticaria(n=2). At the time of presentation range of C-reactive Protein was 4,13 mg/dl to 28mg/dl; PCT in 6 patients (1,92 ng/ml to 21,5ng/ml). 2 patients displayed leukocytosis and 2 leukopenia. All patients received IVIGs. 8 patients received steroids for 4-6 weeks, 4 received ASS. In addition, we report a 12-year-old patient who presented with MIS-C, several days after his second mRNA COVID-19 vaccination. Clinically he presented with polyserositis, severe gastrointestinal symptoms and consequently a Manifestation in multiorgan failure. IgG-Antibodies against spike proteins of SARS-CoV-2 were detected, indicating a successful vaccination, while SARS-CoV-2 Nucleocapsid protein antibodies were negative. Immunosuppression with steroids was initiated. Due to side effects, treatment with steroids and later interleukin 1 receptor antagonists was finally continued with Tacrolimus and Mycophenolic acid leading to improvement and discharge after 79Days. Several autoantibodies against G-protein coupled receptors (GPCR-AAb), previously associated with Long-COVID disease, were detected in a cardiomyocyte bioassay. Follow-up of the patient showed him (2/8/12 weeks) in good condition. AGPCR-AAb remained negative. Therapy was expanded to Omega-3-acid ethyl esters intending to lower risk for autoimmune relapse.
Conclusion
SARS CoV-2 virus exerts multiorgan manifestations due to hyperinflammatory immunological processes. Within two years of the ongoing COVID-19 pandemic within the metropolitan area Nuremberg-Erlangen, we identified ten patients with COVID induced MIS-C, as well as one patient with mRNA vaccination associated MIS-C. Due to the severity of MIS-C, early detection and intervention is warranted. Despite lower in incidence, it developed a serious condition and must be taken into account after vaccination in children without preceding COVID infection.