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Outcomes of MIS-C patients treated with anakinra: a retrospective multicenter national study
Francesco Licciardi1, Carlotta Covizzi1, Marta Dellepiane1
1Division of Pediatric Immunology and Rheumatology, Department of Public Health and Pediatrics, "Regina Margherita" Children Hospital, University of Turin, Turin, Italy.
Insights
Anakinra shows promise for treating severe multisystem inflammatory syndrome in children (MIS-C) unresponsive to standard therapies, with favorable outcomes and few side effects. Further research is needed to confirm IL-1 inhibition
Area of Science:
- Pediatric Rheumatology
- Pediatric Infectious Diseases
- Critical Care Medicine
Background:
- Multisystem inflammatory syndrome in children (MIS-C) unresponsive to IVIG and/or steroids presents treatment challenges.
- The efficacy of IL-1 receptor antagonist anakinra for severe MIS-C is not well-established.
- Investigating anakinra's role in refractory MIS-C is crucial for optimizing patient care.
Purpose of the Study:
- To evaluate the clinical effectiveness and safety of anakinra in children with MIS-C.
- To assess anakinra's impact on treatment step-up, fever resolution, and C-reactive protein (CRP) reduction.
- To determine the incidence of Coronary Artery Anomalies (CAA) in MIS-C patients treated with anakinra.
Main Methods:
- Retrospective, multicenter study of 35 MIS-C patients treated with anakinra in Italy (Jan 2020-Feb 2021).
- Patients received anakinra intravenously or subcutaneously, often concurrently with corticosteroids and/or IVIG.
- Outcomes included treatment escalation, fever and CRP response, and development of CAA.
Main Results:
- Only 2 out of 35 patients required further treatment escalation after anakinra initiation.
- No patients developed Coronary Artery Anomalies (CAA) during the follow-up period.
- The most frequent side effect was elevated ALT (17.1%), with generally favorable safety profile.
Conclusions:
- Anakinra demonstrated favorable clinical outcomes and a low incidence of side effects in severe MIS-C patients.
- The concurrent use of steroids and IVIG complicates definitive conclusions on the necessity of IL-1 inhibition.
- Anakinra represents a potential therapeutic option for refractory MIS-C, warranting further investigation.
Background:
The treatment of multisystem inflammatory syndrome in children unresponsive to first-line therapies (IVIG and/or steroids) is challenging. The effectiveness of IL-1 receptor antagonist, anakinra, is debated.
Patients And Methods:
We conducted an anonymous retrospective multicenter study on MIS-C patients treated with anakinra in Italy from January 2020 to February 2021. Our study outcomes included the percentage of patients who required further therapeutic step-up, the percentage of patients who experienced fever resolution within 24 h and a reduction of CRP by half within 48 h, and the percentage of patients who developed Coronary Artery Anomalies (CAA) during follow-up.
Results:
35 cases of MIS-C were treated in 10 hospitals. Of these, 13 patients started anakinra while in the ICU, and 22 patients started anakinra in other wards. 25 patients (71.4%) were treated with corticosteroids at a starting dose 2-30 mg/Kg/day plus IVIG (2 g/Kg), 10 patients (28.6%) received only corticosteroids without IVIG. Anakinra was administered intravenously to all patients in Group A (mean dose 8 mg/Kg/day), and subcutaneously in Group B (mean dose 4 mg/Kg/day). Only two patients required further treatment step-up and no patients developed CAA after receiving anakinra. The most commonly observed side effect was an increase in ALT, occurring in 17.1% of patients.
Conclusions:
In this retrospective cohort of severe MIS-C patients treated with anakinra we report favorable clinical outcomes with a low incidence of side effects. The simultaneous use of steroids ± IVIG in these patients hinders definitive conclusions regarding the need of IL-1 inhibition in MIS-C treatment.
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