Are Angiotensin Converting Enzyme and von Willebrand Factor Circulating Levels Useful Surrogate Parameters to Monitor Disease Activity in Kawasaki Disease?

To verify if Angiotensin Converting Enzyme (ACE) and von Willebrand factor (vWF) may be used as a laboratory marker for the follow-up of endothelial derangement and therapeutic efficacy in Kawasaki disease (KD), circulating ACE, vWF routine hematological tests and cardiac involvement were assessed in 32 children with established diagnosis of KD before and up to six months after intravenous γ-globulins (IVIG) treatment. IVIG treatment normalized progressively all the hematological parameters to levels comparable with healthy controls within 30 days. At baseline, ACE levels resulted significantly lower (1.8 ± 1.3 pmol/ml/min), and vWF levels significantly increased (210.3 ± 35.2%) when compared with controls (respectively 7.0 ± 0.9 pm/ml/min and 99 ± 17.9%). Seven days after the treatment vWF levels were decreased (188 ± 18.4%) but still significantly higher than controls, and fully normalized after 15 days (104.8 ± 14.3%). ACE levels were found progressively increased at 7, 15, and 30 days after the treatment (respectively 2.7 ± 1.0, 3.7 ± 0.4, 5.04 ± 0.9 pm/ml/min) and reached the range of normality only after two months (7.74 ± 2.46 pm/ml/min). The present study shows that ACE and vWF circulating levels are significantly modified during the acute phase of the disease.