Treatment Intensification in Patients With Kawasaki Disease and Coronary Aneurysm at Diagnosis
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Published on: February 3, 2021
Audrey Dionne1,2, Jane C Burns3, Nagib Dahdah4
1Department of Cardiology and.
Insights
Kawasaki disease can cause coronary artery aneurysms (CAA). Adding corticosteroids or infliximab to intravenous immunoglobulin (IVIg) therapy in high-risk children with CAA may reduce aneurysm size progression.
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Immunology
Background:
- Coronary artery aneurysms (CAA) are a significant complication of Kawasaki disease.
- Intravenous immunoglobulin (IVIg) therapy initiated within 10 days of fever onset significantly reduces CAA risk.
- The long-term effects of adjunctive corticosteroids or infliximab in high-risk CAA patients are not well-established.
Purpose of the Study:
- To evaluate the impact of adjunctive corticosteroids or infliximab with IVIg on coronary artery aneurysm (CAA) size progression in children with Kawasaki disease.
- To compare the effectiveness of IVIg monotherapy versus combination therapy (IVIg with corticosteroids or infliximab) in managing CAA.
Main Methods:
- Retrospective multicenter study involving children diagnosed with CAA (z score ≥2.5 and <10).
- Patients received primary therapy with IVIg alone, or in combination with corticosteroids or infliximab, within 10 days of fever onset.
- Analysis focused on changes in coronary z scores during follow-up.
Main Results:
- No significant difference in median coronary z scores at diagnosis among the three treatment groups (IVIg alone, IVIg + corticosteroids, IVIg + infliximab).
- Both corticosteroids and infliximab, when used as primary treatment intensification with IVIg, were independent protective factors against the progression of coronary artery size.
- Patients receiving adjunctive therapy showed less progression in CAA size compared to those receiving IVIg alone.
Conclusions:
- In high-risk Kawasaki disease patients with baseline CAA, adding corticosteroids or infliximab to IVIg therapy may limit aneurysm size progression.
- Further prospective randomized trials are necessary to establish optimal adjunctive treatment strategies for patients presenting with CAA.
Background:
Coronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to <5%. Corticosteroids and infliximab are often used in high-risk patients or those with CAA at diagnosis, but there are no data on their longer-term impact on CAA.
Methods:
Retrospective multicenter study including children who had CAA with a z score ≥2.5 and <10 at time of diagnosis and who received primary therapy with IVIg alone or in combination with either corticosteroids or infliximab within 10 days of onset of fever.
Results:
Of 121 children, with a median age of 2.8 (range 0.1-15.5) years, 30 (25%) received primary therapy with corticosteroids and IVIg, 58 (48%) received primary therapy with infliximab and IVIg, and 33 (27%) received primary therapy with IVIg only. Median coronary z scores at the time of diagnosis did not differ among treatment groups (P = .39). Primary treatment intensification with either corticosteroids or infliximab were independent protective factors against progression of coronary size on follow-up (coefficient: -1.31 [95% confidence interval: -2.33 to -0.29]; coefficient: -1.07 [95% confidence interval: -1.95 to -0.19], respectively).
Conclusions:
Among a high-risk group of patients with Kawasaki disease with CAA on baseline echocardiography, those treated with corticosteroids or infliximab in addition to IVIg had less progression in CAA size compared with those treated with IVIg alone. Prospective randomized trials are needed to determine the best adjunctive treatment of patients who present with CAA.
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