The American College of Rheumatology (ACR) recently released guidance for the clinical management of multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory coronavirus 2 (SARS-CoV-2) and hyperinflammation in coronavirus disease 2019 (COVID-19). Developed by the ACR MIS-C and COVID-19 Related Hyperinflammation Task Force, these guidelines include the diagnostic evaluation and treatment of SARS-CoV-2-associated MIS-C and hyperinflammation in children with COVID-19. The full report is published on the ACR website.

ACR Recommendations for the Management of MIS-C

General Statements

The ACR task force noted that MIS-C is a relatively rare complication of infection with SARS-CoV-2, with a majority of children with COVID-19 presenting with mild symptoms and showing excellent outcomes.

Because MIS-C is temporally associated with SARS-CoV-2 infection, the prevalence of the virus may change over time, and thus, clinical decisions should be taken accordingly.

Diagnostic Evaluation of MIS-C

The task force has recommended that a child with suspected SARS-CoV-2-associated MIS-C should receive testing for other infectious and noninfectious etiologies, too. These patients may receive additional diagnostic tests, including those of the chest, abdomen, and/or central nervous system.

Suggestive symptoms of MIS-C include rash, gastrointestinal symptoms, oral mucosal changes, conjunctivitis, and neurologic symptoms. In patients with stable vital signs and good physical examinations, outpatient care, along with follow-up care, may be considered. However, most patients with suspected MIS-C should be hospitalized, particularly if they present with abnormal vital signs; respiratory distress of any severity; neurologic deficits; renal or hepatic injury; elevated inflammatory markers (C-reactive protein [CRP], ≥10.00 mg/dL); or abnormal electrocardiogram (EKG), B-type natriuretic peptide (BNP), or troponin T. Patients who present with shock, respiratory distress, neurologic changes (including altered mental status, encephalopathy, papilledema), or features of Kawasaki disease must be hospitalized for further evaluation, regardless of MIS-C status.

Patients with suspected MIS-C should be managed by a multidisciplinary team of pediatric rheumatologists, cardiologists, infectious disease specialists, and hepatologists. Consultation with other specialties may also be considered.

Distinguishing MIS-C From Kawasaki Disease

Patients with Kawasaki disease not associated with SARS-CoV-2 should continue to receive standard care.

Although MIS-C and Kawasaki disease may have overlapping clinical features, including conjunctival injection, red/cracked lips, strawberry tongue, and erythematous hands and feet, the task force has suggested several distinguishing epidemiologic and clinical features between the 2 conditions. Some of them include increased incidence of MIS-C in patients of African, Afro-Caribbean, and Hispanic descent; decreased incidence of MIS-C in patients of East Asian descent; broader age range and more prominent gastrointestinal and neurologic symptoms in patients with MIS-C vs Kawasaki disease; lower platelet counts, lower absolute lymphocyte counts, and higher CRP levels in patients with MIS-C.

This article originally appeared on Rheumatology Advisor