Coronary Artery Complication in Kawasaki Disease and the Importance of Early Intervention : A Systematic Review and Meta-analysis

JAMA Pediatr. 2016 Dec 1;170(12):1156-1163. doi: 10.1001/jamapediatrics.2016.2055.


Importance: The timing and selection of patients with Kawasaki disease for corticosteroid use to prevent coronary artery complications remain controversial.

Objective: To evaluate the effect of corticosteroid therapy in KD.

Data sources: Databases of Medline, The Cochrane Library, and the website until July 2015. We used the key words ["Kawasaki disease"] and ["steroid" OR "corticosteroid"] to retrieve potentially relevant studies in the databases of Medline, the Cochrane Library, and the website until July 2015. Both English and non-English literature was identified. Titles and abstracts were reviewed by 2 authors (S.C. and Y.D.) to determine suitability for inclusion. Relevant articles were reassessed by reviewing the full text. Discrepancies in study inclusion were resolved by consensus (M.G.K.).

Study selection: Clinical studies that compared corticosteroids plus intravenous immunoglobulin (IVIG) therapy with IVIG therapy alone in treating patients with KD. Studies either using corticosteroids as initial therapy or as rescue therapy were included.

Data extraction and synthesis: Investigators independently extracted the data information. Data were quantitatively synthesized using random-effects analysis.

Main outcomes and measures: Rate of coronary artery abnormalities.

Results: Sixteen comparative studies characterizing 2746 patients were analyzed. The duration of illness before corticosteroids therapy was significantly shorter in the initial corticosteroids subset than in the rescue corticosteroids subset. The rate of coronary artery abnormalities was significantly lower in adjunctive corticosteroids therapy than in IVIG therapy (odds ratio [OR], 0.424; 95% CI, 0.270-0.665). Meta-regression based on known variables demonstrated that the overall efficacy was negatively correlated with the duration of illness before corticosteroid therapy (P < .001). Subgroup analysis, including studies using corticosteroids plus IVIG as initial therapy, showed a more advantageous effect than IVIG alone regarding coronary artery abnormality prevention (OR, 0.320; 95% CI, 0.183-0.560), whereas this benefit was not found in a subgroup of studies using corticosteroids as rescue therapy. Further analysis found that patients predicted at baseline to be at high risk of IVIG resistance seemed to obtain the greatest benefit from adjunctive corticosteroid therapy regarding coronary artery abnormality prevention (OR, 0.240; 95% CI, 0.123-0.467). The fever duration was significantly reduced in the corticosteroids group. The favorable effects of corticosteroids were conferred without an increased risk of adverse events.

Conclusions and relevance: This study highlights the importance of timing to prevent coronary artery complication in treating KD. High-risk patients with KD benefit greatly from a timely and potent adjunctive corticosteroid therapy strategy.

Publication types

  • Meta-Analysis
  • Review
  • Systematic Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Chemotherapy, Adjuvant
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Coronary Artery Disease / prevention & control*
  • Drug Therapy, Combination
  • Female
  • Fever / prevention & control
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Infant
  • Infant, Newborn
  • Male
  • Mucocutaneous Lymph Node Syndrome / drug therapy*
  • Risk Factors
  • Time-to-Treatment
  • Treatment Outcome


  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous