The role of corticosteroids in the treatment of Kawasaki disease

ABSTRACT

Introduction: Kawasaki disease (KD) is a form of systemic vasculitis that can lead to complications of coronary artery lesions (CAL). Diagnosis without delay and treatment with intravenous immunoglobulin (IVIG) are vital for a better prognosis. Anti-inflammatory drugs are generally used empirically in pediatric patients as off label. Corticosteroids are effective with anti-inflammatory effects applied in vasculitis.

Areas covered: The timing of corticosteroid treatment in KD has been widely discussed by scholars. Some corticosteroids may still be effective which could be useful for such specific populations as high-risk patients. In this narrative review, we searched clinical studies, meta-analyzes, and systemic reviews using the PubMed database to summarize the available evidence on corticosteroid usage in KD through October 2019 and then discussed the relevant issues.

Expert opinion: Today, the available evidence is more powerful to recommend corticosteroids for KD, moving from an unproven therapy to an effective adjunctive treatment. We suggest using methylprednisolone pulse therapy as an alternative rescue therapy for immunoglobulin-resistant KD, as well as identifying high-risk patients who need initial corticosteroid with IVIG treatment with an adequate route, dose, and duration. In the future, studies that evaluate the precision role of corticosteroids for individualized KD patients with CAL are warranted.

KEYWORDS:

• Coronary artery lesions
• corticosteroid
• IVIG resistant
• Kawasaki disease
• methylprednisolone pulse

Article highlights

• We suggest using methylprednisolone pulse therapy as the alternative of rescue therapy for immunoglobulin-resistant KD.

• Studies for predicted IVIG non-responders suggested that patients with KD treated with high-dose IVIG and corticosteroids reduce the risk of CAL in Japan.

• We found corticosteroid to have an effective role in treating KD in the time after or together with IVIG treatment but not before prescription of IVIG.

• Some case reports have indicated anti-inflammatory effects of pulse intravenous MP for possible life-threatening complications of KD.

• Future studies are warranted to examine the risk scores with the aid of potential biomarkers to tailor treatment for individual patients with Kawasaki disease. The timing and selection of particular corticosteroids require careful consideration in KD therapy. Longer-term and large-scale studies of side effects are needed

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was funded by grants from the Chang Gung Memorial Hospital [CMRPG8H1161] and the National Science Council, Taiwan [MOST 108-2314-B-182 −037 -MY3]. The funders had no role in the study design, data collection and analysis, decision to publish, or preparation of this manuscript.