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Primary adjunctive corticosteroid therapy is associated with improved outcomes for patients with Kawasaki disease with coronary artery aneurysms at diagnosis
  1. Kevin G Friedman1,
  2. Kimberlee Gauvreau2,3,
  3. Annette Baker2,4,
  4. Mary Beth Son5,
  5. Robert Sundel4,6,
  6. Audrey Dionne7,
  7. Thomas Giorgio8,
  8. Sarah De Ferranti7,
  9. Jane W Newburger1
  1. 1Boston Children's Hospital—Pediatric Cardiology, Harvard Medical School, Boston, Massachusetts, USA
  2. 2Cardiology, Boston Children’s Hospital, Boston, Massachusetts, USA
  3. 3Harvard Medical School, Boston, Massachusetts, USA
  4. 4Pediatrics, Harvard Medical School, Boston, Massachusetts, USA
  5. 5Children’s Hospital, Boston, Massachusetts, USA
  6. 6Medicine, Children’s Hospital Boston, Boston, Massachusetts, USA
  7. 7Boston Children’s Hospital, Boston, Massachusetts, USA
  8. 8Boston Children’s Hospital—Pediatric Cardiology, Boston Children’s Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr Kevin G Friedman, Boston Children's Hospital—Pediatric Cardiology, Harvard Medical School, Boston, MA 02115, USA; kevin.friedman{at}cardio.chboston.org

Abstract

Objective Patients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outcomes in patients with CAA at diagnosis.

Design Single-centre, retrospective review.

Patients Patients with KD diagnosed within 10 days of fever onset and with baseline CA z-score ≥2.5.

Interventions Primary treatment with IVIG (n=162) versus IVIG plus corticosteroids (n=48).

Main outcome measures Treatment resistance (persistent fever >36 hours after initial treatment), CAA regression rate.

Results Of the 92 patients with KD who received corticosteroids at our institution from 2012 to 2019, 48 met the inclusion criteria for primary adjunctive therapy. The corticosteroid group was younger and had larger baseline CAAs compared with historical controls. Demographics and laboratory values were otherwise similar between groups. The corticosteroid group had a less treatment resistance (4% vs 30%, p=0.003) and a greater improvement in C reactive protein. After adjusting for baseline CA z-score, age and baseline bilateral versus unilateral CAA, the corticosteroid group had a higher odds of (OR 2.77 (1.04, 7.42), p=0.042) and a shorter time to CAA regression (HR 1.94 (1.27, 2.96), p=0.002).

Conclusion Primary adjunctive corticosteroid therapy is associated with decreased initial treatment resistance, greater improvement in inflammatory markers and higher likelihood of CAA regression in patients who have CAA at diagnosis. Multi-centre, randomised controlled trials are needed to confirm the benefits of corticosteroids in patients with CAA at diagnosis and to compare corticosteroids with other adjunctive therapies.

  • cardiology
  • therapeutics
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Footnotes

  • Correction notice This paper has been corrected since it was published online. The last author's surname was spelled incorrectly.

  • Contributors KGF, KG, AB, MBS, RS, AD, TG, SDF, JWN: substantial contributions to the conception of the work, data acquisition, analysis, and interpretation; drafting and revising the work for important intellectual content, final approval of the manuscript submitted.

  • Funding This work was supported by The McCance Family Foundation and Gordon and Marilyn Macklin Foundation.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval Institutional Review Board approval was obtained (IRB #M06-09-0436).

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data will be made available on reasonable request.

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