Approach to immunomodulatory therapy in multisystem inflammatory syndrome in children (MIS-C)

This algorithm summarizes our approach to the choice of immune-modifying therapy for pediatric patients hospitalized for COVID-19-associated MIS-C (also called pediatric inflammatory multisystem syndrome [PIMS]). Most children who meet diagnostic criteria for MIS-C are managed in the inpatient setting. It may be reasonable to manage select patients with mild symptoms in the outpatient setting, provided that the child is well-appearing and close clinical follow-up can be assured. Management of mildly affected patients varies from center to center and is beyond the scope of this algorithm. This algorithm is intended for use in conjunction with additional UpToDate content. Refer to UpToDate topics on MIS-C for discussion of the diagnostic criteria and for additional details regarding management, including discussion of the evidence supporting our treatment recommendations.

IVIG: intravenous immune globulin; COVID-19: coronavirus disease 2019; MIS-C: multisystem inflammatory syndrome in children; SARS-CoV-2: severe acute respiratory syndrome coronavirus 2; CRP: C-reactive protein; IL: interleukin; MAS: macrophage activation syndrome.

* Glucocorticoid therapy is initially given as intravenous methylprednisolone 2 mg/kg/day (maximum 60 to 80 mg/day) in 2 divided doses. Once the patient has defervesced and is improved clinically, this can be transitioned to an equivalent oral dose of prednisolone or prednisone (maximum dose 60 mg/day) by the time of discharge and then tapered off over 2 to 3 weeks. Preexisting conditions that may warrant avoidance of glucocorticoids in the patient with mild MIS-C include diabetes mellitus, hypertension, and obesity.

¶ The dosing for IVIG in this setting is 2 g/kg (maximum 100 g) administered in a single infusion over 8 to 12 hours. For children with obesity, dosing is based on ideal body weight. For patients with significant cardiac dysfunction, if there is concern that the patient will not tolerate the volume load of the full dose in a single infusion, it can be given in divided doses over 2 days. Patients should have blood drawn for serologic testing for SARS-CoV-2 and other pathogens prior to administration of IVIG.

Δ An adequate response is signaled by resolution of fevers, improvement in inflammatory markers (eg, CRP, ferritin), and improvement in clinical status (eg, resolution of shock [if present], improvement in cardiac function [if initially depressed], regression of coronary artery findings [if present]). Patients with persistent or recurrent fevers, persistently elevated or rising inflammatory markers, and/or worsening clinical status are considered to have an inadequate response. Assessment of the patient's response to initial therapy is typically made over 1 to 3 days; however, it may be reasonable to wait a longer duration in patients without severe manifestations.

◊ The most common adjunctive therapies are high-dose (pulse) glucocorticoids, the IL-1 inhibitor anakinra, and the tumor necrosis factor inhibitor infliximab. Infliximab should not be used in patients with signs of MAS. Additional adjunctive therapies include other IL-1 inhibitors (eg, canakinumab) and IL-6 inhibitors (eg, tocilizumab). The benefits and risks of these therapies in children with MIS-C are uncertain. Consultation with pediatric infectious disease and rheumatology specialists is advised.

§ Complications can include coronary artery dilation/aneurysm, cardiac dysfunction, and arrhythmia. For details regarding monitoring for patients with MIS-C, including our suggested schedule for follow-up echocardiography, refer to UpToDate topics on MIS-C.

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Approach to immunomodulatory therapy in multisystem inflammatory syndrome in children (MIS-C)