| Abstract | Multisustavni upalni sindrom u djece, također poznat i kao MIS-C, rijetka je, ali ozbiljna bolest koja se može pojaviti oko mjesec dana nakon što dijete preboli COVID-19 ili je bilo izloženo SARS-CoV-2. MIS-C uzrokuje upalu više organa i organskih sustava koja se manifestira visokom perzistentnom vrućicom s mogućim zatajenjem organa zbog šoka koji nastaje uslijed vaskulitisa. Drugi simptomi i znakovi ovog sindroma mogu biti mučnina, povraćanje, proljev, bol u abdomenu, letargija, bol u vratu te osip. S obzirom na širok raspon izraženih simptoma i njihovih intenziteta, diferencijalna dijagnoza multisustavnog upalnog sindroma u djece je opsežna pa je potrebno isključiti teški akutni COVID-19, bakterijsku sepsu, sindrom toksičnog šoka, apendicits, Kawasakijevu bolest, sistemski eritematozni lupus, sindrom aktivacije makrofaga te infekcije drugim virusima ili rikecijama. Uzrok sindroma i dalje je nepoznat, ali poznato je da su se grupe novih slučajeva pojavljivale dva do šest tjedana nakon vršne incidencije zaraze populacije SARS-CoV-2. MIS-C patofiziološki je sličan Kawasakijevoj bolesti i sindromu aktivacije makrofaga. Sva su tri stanja hiperinflamatorna, što znači da postoji disregulacija imunosnog odgovora. Terapija multisustavnog upalnog sindroma u djece sastoji se od niskih doza metilprednizolona i intravenskih imunoglobulina (IVIG), ako ne postoje znakovi zahvaćenosti miokarda. Ako postoje, djetetu se ne daje IVIG budući da dodatno volumno opterećuju srce, već se primjenjuju visoke pulsne doze metilprednizolona uz drugu liniju terapije koju čini subkutano aplicirana anakinra, antagonist receptora za interleukin 1. Unatoč sličnostima mehanizama prirođenih grešaka imuniteta koje uzrokuju autoimunosne i autoinflamatorne bolesti i MIS-C-a, njihova povezanost do sada nije potvrđena. |
| Abstract (english) | Multisystem inflammatory syndrome in children, also known as MIS-C, is a rare but serious illness that can appear around a month after a child recovers from COVID-19 or has been exposed to SARS-CoV-2. MIS-C causes inflammation in multiple organs and organ systems and is manifested by persistent high fever with possible organ failure due to shock caused by vasculitis. Other signs and symptoms of this syndrome can be nausea, vomiting, diarrhea, abdominal pain, lethargy, neck pain and rash. Considering the wide range of expressed symptoms and their intensities, the differential diagnosis of multisystem inflammatory syndrome in children is extensive, so it is necessary to exclude severe acute COVID-19, bacterial sepsis, toxic shock syndrome, appendicitis, Kawasaki disease, systemic lupus erythematosus, macrophage activation syndrome, infections with other viruses or rickettsiae. The cause of the syndrome is still unknown, but it is known that clusters of new cases appeared 2 to 6 weeks after the peak incidence of SARS-CoV-2 infection in the population. In its pathophysiology, MIS-C is similar to Kawasaki disease and macrophage activation syndrome, and all three conditions are hyperinflammatory, meaning there is a dysregulation of the immune response. Therapy of multisystem inflammatory syndrome in children consists of low doses of methylprednisolone and intravenous immunoglobulins if the child isn’t showing any signs of myocardial involvement. If so, the child is not given IVIG because those put an additional volume load on the heart. Instead, high pulse doses of methylprednisolone are applied with subcutaneously applied anakinra, an antagonist of the receptor for interleukin 1, as step-up therapy. Despite the similarity of the mechanism of inborn errors of immunity that cause autoimmune and autoinflammatory disease and MIS-C, their association has not been confirmed so far. |
