Hi,

my first question is how old is the child, and at what day of fever period it is. You may know, that in regard to the cardiovascular involvement, the child may first develop signs of myocarditis and later around 14 to 40 days coronary abnormalities in terms of aneurysmatic changes, which are not always to be seen in echocardiography.

If you have a very strong suspicion of Kawasaki disease and the child is quite young you should absolutely consider to give the recommended treatment of ASS and IVIGs, because fever is normally declined when cardiovascular changes occur.

Please can you give me more information about your patient.

Best

Hi, thank you for your early response.

The question is for children who fulfill incomplete Kawasaki disease criteria and experience spontaneous defervescence at day 5-10 of fever spontaneously without IGIV administration, joined to a decrease of levels of acute phase reactants and normal echocardiographic evaluation during this period. 

I think that these patients could have another condition that mimics Kawasaki or a Kawasaki disease with an indolent course, and no benefits of IGIV are got because if it occurs it means that no inflammation in present at this moment, and i think that the major indication of IGIV is to stop the inflammatory state that lead to coronary lesions.

My current practice is not to administer IGIV and perform a close weekly follow-up for 2 months, and if echocardiographic lesions or elevation of acute phase reactants or fever are detected, then i administer IGIV. 

In my experience, this rare subgroup of patients have lower levels of NT-proBNP, and no  develop important coronary sequelae at 6 months follow-up.

Thank you for your opinion.

Best

Hi,

you might be absolutely right in terms of a Kawasaki mimiking other inflammatory disease, but nevertheless it remains, that only a limited number of coronary artery alterations are visible by echo and the age of the patient should be taken into account. The younger they are the more likely they develop coronary disease. Furthermore, as being a pediatric cardiologist (as you I suggest) we are thinking of the consequences of our therapies  far beyond the childhood period. I am personally aware of a patient who developed acute coronary disease at 22 years of age with a history of Kawasaki disease without obvious coronary artery aneurysms complicating Kawasaki disease with otherwise empty history. Could that be related to chronic alterations of the coronary arteries 20 years after an inflammatory process?

Best

Hi, 

In response to the case that you expose i think that the risk to develop coronary complications in adult life is rare if there is not any coronary detectable lesion 6 month after the acute phase of the Kawasaki disease. In the reference that i share the authors exposed their experience about longer-term rates of adverse cardiac events in kawasaki disease. This study despite its limitations, showed low rates of long-term cardiovascular complications over an average follow-up period of ∼15 years after the KD illness. Only a minority (5%) of patients developed persistent coronary aneurysm after the acute KD illness, and long-term complications were restricted to this subgroup.

Back to my topic i agree that is necessary to treat with IGIV if i suspect Kawasaki disease and the age is a major factor to take in account. But in my opinion i think that there is an overuse of IGIV due to the fear to be late, that can be avoided if we are more selective with its indication with patients such as the example i said. I think that in this situation (spontaneous defervescence before 7-10 days of fever), conditions mimicking  kawasaki are more frequent conditions that kawasaki disease. 

However i know that it is a rare situation and I therefore ask the experience of anyone   with this subject.

Best