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A Case of Severe Multisystem Inflammatory Syndrome in Children (MIS-C) Treated with Multiple Biologics
Beenish Zulfiqar1, Hira Imran1, Kathleen Collins1
1University of Tennessee Memphis, Department of Rheumatology, Memphis, USA.
Insights
Multisystem inflammatory syndrome in children (MIS-C) is a severe, delayed COVID-19 complication causing cytokine storms. Early diagnosis and immunomodulatory treatment are crucial for preventing organ damage and mortality.
Area of Science:
- Pediatric critical care medicine
- Infectious disease immunology
- Viral pathogenesis
Background:
- COVID-19 can trigger delayed autoinflammatory responses, including cytokine storms.
- Multisystem inflammatory syndrome in children (MIS-C) is a serious post-infectious complication.
- MIS-C presents with diverse symptoms and significant laboratory abnormalities.
Observation:
- A 12-year-old boy presented with severe MIS-C symptoms, requiring intensive life support including intubation, pressors, ECMO, and renal replacement therapy.
- The patient exhibited classic signs and symptoms of severe MIS-C.
- Lab results showed elevated acute phase reactants and cardiac markers.
Findings:
- Successful treatment involved a combination of immunomodulatory therapies.
- Key treatments included intravenous immune globulin (IVIG), steroids, and targeted cytokine inhibitors (IL-6, TNF-a, IL-1).
- Janus kinase (JAK) inhibitors were also utilized in the treatment regimen.
Implications:
- This case highlights the critical need for early recognition and aggressive management of MIS-C.
- Comprehensive immunomodulatory therapy can lead to successful outcomes in severe MIS-C cases.
- Understanding MIS-C's pathophysiology is vital for developing effective treatment strategies and preventing long-term sequelae.
Abstract:
The COVID-19 virus has impacted global health on a wide scale, affecting humans of all ages and ethnicities. While most have mild upper respiratory viral symptoms, some have died due to severe pneumonia, acute respiratory distress syndrome (ARDS), or coagulopathies to mention a few. It has been postulated that the COVID-19 virus can initiate an autoinflammatory reaction in the body via multiple pathways of cytokine activation. The virus can cause delayed response after 4-8 weeks of acute infection, which resembles a cytokine storm or MAS (macrophage activation syndrome). This highly inflammatory syndrome, called MIS-C or multisystem inflammatory response syndrome, needs to be diagnosed and treated early to prevent multiorgan damage and mortality. There are widespread lab abnormalities including highly elevated acute phase reactants ferritin, D-Dimer, lactate dehydrogenase (LDH), creatinine kinase (CK), sedimentation rate (ESR), and C-reactive protein (CRP) as well as markers of cardiac damage including troponin and brain natriuretic peptide (BNP). The syndrome can present in unique ways from classic MIS-C with hypovolemic shock to Kawasaki disease-like presentation. We present a case of a 12-year-old boy who presented to Le Bonheur Children's Hospital in Memphis with classic signs and symptoms of "severe" MIS-C requiring intubation, multiple pressors, ECMO, and renal replacement therapy. He was treated successfully with immunomodulating medicines including intravenous immune globulin (IVIG), steroids, interleukin-6 inhibitor, tumor necrosis factor-a inhibitor, interleukin-1 inhibitor, and Janus kinase inhibitor.
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