. Author manuscript; available in PMC: 2022 Sep 1.

Published in final edited form as: Curr Opin Rheumatol. 2021 Sep 1;33(5):378–386. doi: 10.1097/BOR.0000000000000818

Table 2.

Treatment Guidelines

	American College of Rheumatology (USA)⁵⁵	PIMS-TS National Consensus Management Study Group (UK)⁵⁴
Date Published	June 2020, Revised November 2020	September 2020

Population Applied To	Children with MIS-C	Children with PIMS-TS and Kawasaki disease-like phenotype	Children with PIMS-TS and non-specific phenotype^*

IVIG	2 g/kg based on ideal body weight -First-line therapy in hospitalized MIS-C patients -Second dose of IVIG not recommended	2 g/kg dosed on ideal body weight - First-line therapy in all PIMS-TS with KD-like phenotype and in all treated non-specific PIMS-TS patients - Second dose considered for children who have not responded to the first dose
Glucocorticoids	IV methylprednisolone (1–2 mg/kg/day) - Fist-line with IVIG if shock or organ threatening disease - Second-line in refractory disease in other children IV methylprednisolone (10–30 mg/kg/day) - For treatment intensification in refractory disease 2–3 week steroid taper to prevent rebound	IV methylprednisolone (10–30 mg/kg/day) - First-line with IVIG if < 12 months or coronary artery abnormalities - As second-line in other children	IV methylprednisolone (10–30 mg/kg/day) -Second-line therapy
Additional Immunomodulation	High-dose anakinra if refractory to IVIG and/or steroids	Infliximab if non-responsive to IVIG and steroids	Third line if non-responsive to IVIG and steroids Consensus not reached; equipoise for tocilizumab, anakinra, and infliximab
Anticoagulation	-Low dose ASA in all MIS-C patients without significant bleeding risk until normalization of plt count and confirmed normal coronary arteries. -Anticoagulation if CAA with z-score≥10, documented thrombosis, or Or EF<35%	- If >12 years, should wear compression stockings - Low-dose ASA for minimum 6 weeks in all patients - Local protocol for management of a thrombotic event - Consult with hematologist re: long-term antiplatelet and anticoagulation therapy if CAA
Anticoagulation		Local protocol for KD ASA dosing
Antimicrobial	Not addressed	If SARS-CoV-2 positive (RT-PCR or antigen), consider remdesivir IV antibiotics in all patients; should be focused or stopped on the basis of the clinical picture and culture results If criteria for toxic shock syndrome met, clindamycin in addition to broad-spectrum antibiotics

Treatment for this group is recommended in patients who have a coronary abnormality, TSS, progressive disease, or fever > 5 days.