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.