Using steroids to treat Kawasaki disease

Review question

We reviewed the use of a set of drugs known as steroids in children affected by Kawasaki disease for the reduction in the chance of future heart problems as well as the effect on the duration of fever, signs of infection in the blood and the number of days spent in hospital.

Background

We currently have a limited understanding of Kawasaki disease and how best to manage it. This is important as one of the long-term consequences can involve the heart, putting the child at higher risk of life-shortening outcomes.

Study characteristics

Evidence is current to November 2016. Male and female children diagnosed with Kawasaki disease were included in this review. We selected only randomised clinical trials. Trials compared the use of steroids against not using steroids. This review involves seven trials and 922 participants.

Key results

Steroids appear to reduce the risk of heart problems after Kawasaki disease without causing any important side effects. They also reduce the length of symptoms (fever and rash), length of hospital stay, and blood markers associated with being unwell. Certain groups, including those based in Asia, those with higher risk scores, and those receiving longer steroid treatment, may have greater benefit from steroid use, especially with decreasing rates of heart problems, but more tests are needed to answer these questions. More tests are also needed to obtain a more accurate marker of the risk of serious side effects and to determine if there is a lower chance of death when using steroids. Evidence presented in this review suggests that treatment with a long course of steroids should be considered for all children diagnosed with Kawasaki disease until further studies are performed.

Quality of the evidence

Evidence quality was graded according to the GRADE system. Evidence was considered high quality for serious adverse events, mortality and time for laboratory parameters to normalise. Evidence was considered moderate quality for the risk of future heart problems, duration of clinical symptoms (fever, rash) and length of hospital stay. This means that we are reasonably confident that the true effect is close to that estimated in this work.

Authors' conclusions: 

Moderate-quality evidence shows that use of steroids in the acute phase of KD can be associated with improved coronary artery abnormalities, shorter duration of hospital stay and a decreased duration of clinical symptoms. High-quality evidence shows reduced inflammatory marker levels. There were insufficient data available regarding incidence of adverse effects attributable to steroids, mortality and long-term (> 1 year) coronary morbidity. Certain groups, including those based in Asia, those with higher risk scores, and those receiving longer steroid treatment may have greater benefit from steroid use, especially with decreasing rates of heart problems, but more tests are needed to answer these questions. Evidence presented in this study suggests that treatment with a long course of steroids should be considered for all children diagnosed with KD until further studies are performed.

Read the full abstract...
Background: 

Kawasaki disease (KD), or mucocutaneous syndrome, is the leading cause of childhood-acquired heart disease in the developed world. There is much controversy on how best to treat children with KD and in particular who may benefit from additional treatment beyond the standard intravenous immunoglobulin (IVIG) and aspirin, such as the addition of corticosteroids.

Objectives: 

To assess the impact of corticosteroid use on the incidence of coronary artery abnormalities in KD as either first-line or second-line treatment. Corticosteroids may be given alone or in conjunction with other accepted KD treatments. Secondary objectives include the effect of steroids on mortality, the time taken for laboratory parameters to normalise, the duration of acute symptoms (such as fever), the long-term impact of steroid use and evaluating their safety in KD and their efficacy in relevant population subgroups.

Search strategy: 

The Cochrane Vascular Information Specialist searched Cochrane Vascular's Specialised Register (25 November 2016) and the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 10) in the Cochrane Library (searched 25 November 2016). Trial registries were also searched for details of ongoing or unpublished studies.

Selection criteria: 

We selected randomised trials involving children with all severities of KD who were treated with corticosteroids, including different types of corticosteroid and different durations of treatment.

Data collection and analysis: 

MJS and GMC independently selected studies, assessed evidence quality and extracted data. This process was overseen by AJW.

Main results: 

Seven trials consisting of 922 participants were included in this analysis. Trials ranged from 32 to 242 participants. On pooled analysis, corticosteroids reduced the subsequent occurrence of coronary artery abnormalities (odds ratio (OR) 0.29, 95% confidence interval (CI) 0.18 to 0.46; 907 participants; 7 studies; I² = 55%) without resultant serious adverse events (no events, 737 participants) and mortality (no events, 915 participants). In addition, corticosteroids reduced the duration of fever (mean difference (MD) −1.65 days, 95% CI −3.31 to 0.00; 210 participants; 2 studies; I² = 88%), time for laboratory parameters (erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)) to normalise (MD −2.80 days, 95% CI −4.38 to −1.22; 178 participants; 1 study) and length of hospital stay (MD −1.41 days, 95% CI −2.36 to −0.46; 39 participants; 1 study). No studies detailed outcomes beyond 24 weeks. Subgroup analysis showed some potential groups that may benefit more than others; however, further randomised controlled trials are required before this can be the basis for clinical action.

Evidence quality was graded according to the GRADE system. Evidence was considered high quality for the incidence of serious adverse events, mortality and time for laboratory parameters to normalise. Evidence was considered moderate for the incidence of coronary artery abnormalities due to potential inconsistencies in data geography and patient benefits according to grouping. Evidence was moderate for duration of clinical symptoms (fever, rash) due to potential subjectivity in measurement. Evidence was moderate for length of hospital stay as only one study recorded this outcome. This means that we are reasonably confident that the true effect is close to that estimated in this work.

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