Timely corticosteroid treatment benefits those with Kawasaki disease

Patients with Kawasaki disease who received corticosteroids plus intravenous immunoglobulin (IVIG) therapy had a reduced risk of coronary artery complications compared with IVIG therapy alone, according to a systematic review and meta-analysis.

Adding corticosteroid therapy was associated with a relative risk reduction of 58 percent in coronary artery abnormalities. The benefit was only found when corticosteroids were used as an initial therapy and not as a rescue therapy after IVIG therapy failed.

Lead researcher Shaojie Chen, MD, PhD, of the Shanghai General Hospital and Shanghai Jiao Tong University School of Medicine in China, and colleagues published their results onlien Oct. 17 in JAMA Pediatrics.

Kawasaki disease, which is also known as mucocutaneous lymph node syndrome, affects mostly infants and children. Previous studies showed that approximately 30 percent to 50 percent of patients with Kawasaki disease develop transient coronary artery dilation in the acute stage, while approximately 25 percent have serious coronary artery abnormalities. Although the standard of care is IVIG therapy plus aspirin, 20 percent to 40 percent of patients are resistant to IVIG therapy.

For this analysis, the researchers searched Medline, The Cochrane Library and Clinicaltrials.gov and identified 16 studies published through July 2015 that compared corticosteroids plus IVIG therapy with IVIG therapy alone in treating patients with Kawasaki disease. The studies enrolled a total of 2,746 patients.

The researchers defined an abnormal coronary artery as an internal lumen diameter greater than 3 mm in a child younger than 5 years old or greater than 4 mm in a child 5 years or older. They used two-dimensional echocardiograms to detect coronary abnormalities.

Of the 16 studies, 10 used corticosteroids as an initial treatment, while six used corticosteroids as a rescue treatment after failure with initial IVIG therapy. The duration of illness before corticosteroids therapy was significantly shorter in studies that used corticosteroids as an initial treatment, according to the researchers.

They added that the rate of coronary artery abnormalities was significantly lower in patients who received corticosteroids plus IVIG therapy. A meta-regression analysis found that a longer duration of illness before corticosteroid intervention was negatively associated with the treatment effect on preventing coronary artery abnormalities. The regression analysis included baseline variables such as sample size, mean age, sex and duration of illness before corticosteroid intervention.

Subgroup analyses showed that using corticosteroids plus IVIG helped prevent coronary artery abnormalities better than IVIG alone. However, among the studies using corticosteroids as a rescue therapy, there was no significant benefit to corticosteroid treatment.

The researchers mentioned a few potential limitations of the analysis, including that the studies had different designs, which could have led to potential bias. Most of the studies were conducted in Japan, where there is high awareness of Kawasaki disease, so the results might not apply to other geographic regions.

“This study highlights the importance of timing in treating Kawasaki disease; high-risk patients with Kawasaki disease benefit greatly from a timely adjunctive corticosteroid therapy strategy,” the researchers wrote.