Adriana Tremoulet, MD, reviewed the classic presentation as well as the incomplete disease presentation of Kawasaki disease (KD) at a breakout session Monday morning at HM12.
Clinical KD is an immunologic reaction triggered by a presumed infectious agent in a genetically susceptible host. The clinical outcome, including coronary aneurysm, is also likely genetically pre-determined. Early identification is essential for proper treatment to decrease the risk of coronary artery aneurysms. Most KD patients will have some elevation of biomarkers, including CRP, ESR, CSF pleocytosis, GGT, ALT, platelets, and WBC. Anemia may also be present. There are ongoing trials of potential laboratory analysis panels.
IVIG remains standard first line therapy for KD. IVIG-resistant KD is defined as persistent fever 36 hours after initial IVIG treatment. Twenty-two percent of patients with IVIG-resistant KD will develop coronary artery aneurysms, a rate similar to untreated KD. There are multiple treatment options for IVIG-resistant KD including a second dose of IVIG, infliximab, steroids, plasmapheresis, cyclophosphamide, methotrexate, and cyclosporine.
- Hospitalists should remain vigilant to identify children with acute KD, including atypical or late presentations.
- Treatment options for IVIG-resistant KD patients are available but protocols are still being evaluated for efficacy.
- There is a potential role of biomarkers in diagnosing KD. These include stratification of patients by inflammatory markers in first 10 days of illness that can diagnose incomplete KD in 90% of children.
- Be aware of potential Kawasaki Disease Shock Syndrome, and continue to give IVIG for these patients.
Dr. Hale is a pediatric hospitalist at Floating Hospital for Children, Tufts Medical Center in Boston.