Klinische Pädiatrie: Offener Zugang
Offener Zugang

Abstrakt

Predicting Very Severe Kawasaki Disease Who Does Not Respond to Initial Steroid Combination Therapy: A Prospective Cohort Study

Yukako Yoshikane, Tatsuki Miyamoto, Junichi Hashimoto, Shinichi Hirose, Atsushi Ogawa

Objective: Kawasaki Disease (KD) is an acute systemic vasculitis that can lead to ischemic heart disease resulting from coronary artery aneurysms. Intravenous Immunoglobulin (IVIG) resistance is a major risk factor for the development of Coronary Artery Lesions (CALs). Pulsed or non-pulsed steroids combination therapy as initial treatment for children with high risk KD was listed in the Japanese guideline in 2012. However, some patients with very severe KD do not improve even with the steroid combination therapy. The purpose of this study was to clarify the risk factors for severe KD patients who do not respond to the steroid combination therapy, and to confirm whether they can be predicted in the early stage. Methods: Forty hospitalized KD patients with high scores on all three Japanese prediction scoring systems and who received steroids with IVIG in initial treatment were prospectively recruited. IVIG+steroid-responder group (n=21) required no additional therapy and IVIG+steroid-resistance group (n=19) required additional therapy. Clinical data before and after the initial treatment were compared between each group. Results: Kobayashi and Egami scores before the initial treatment and the neutrophil count 2 days after the initial treatment were higher in IVIG+steroid-resistance group than that in IVIG+steroid-responder group independently. Kobayashi score ≥ 7, 2 points; Egami score ≥ 4, 1 point; and neutrophil count after initial treatment ≥ 11000/μL, 2 points. Patients who had three points or more were likely to need additional treatment (sensitivity 89%, specificity 76%). Conclusions: Predicting very severe KD who does not respond to the steroid combination therapy in the early stage is possible. Our new, original model may help to predict non-responders to the initial steroid combination therapy and prevent coronary artery aneurysms.