Introduction
Kawasaki disease (KD) is an acute vasculitis that is the leading cause of acquired heart disease in children, with approximately 15-20% of patients with KD suffering intravenous immunoglobulin (IVIG) resistance1. For children who do not respond to initial IVIG treatment, repeated IVIG infusion is recommended by many experts2. However, approximately 10% of patients are resistant to both initial and repeated IVIG therapy3, and thus have a higher risk of coronary artery lesions (CALs). Therefore, early identification of both initial and repeated IVIG resistance is of great importance to reduce CALs, and most importantly, lower medical costs.
It has long been known that inflammation triggered by acute infection can lead to activation of the coagulation system by upregulating the expression of cytokines4. In an investigation of critically ill patients, Ogura et al. found that abnormal coagulation was associated with increased systemic inflammatory response syndrome scores5. In addition, the relationship between the activation of the immune system and coagulation system is evident in systemic autoimmune or immune-mediated diseases6. Therefore, the balance between coagulation and the fibrinolytic system may be disturbed during the acute stages of KD, particularly in patients with IVIG resistance, since KD vasculitis is accompanied by an increase in inflammatory cells and cytokines, and IVIG resistance may reflect a more severe inflammatory immune condition. Unfortunately, few reports analyze the correlation between coagulopathy and IVIG resistance in KD.
Therefore, a large prospective cohort study was conducted to assess the predictive validity of the serum coagulation profile, including prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT), international normalized ratio (INR), and fibrinogen, D-dimer, fibrin degradation products (FDP), and antithrombin III (ATIII) levels for identifying patients with KD at risk for both initial and repeated IVIG resistance.