Current perspectives on Kawasaki disease

Abstract

The etiology of Kawasaki disease (KD) remains unknown despite several years of dedicated research in this direction. Recently coronavirus infection and genetic polymorphisms have been implicated. Since first description of the disease there have been few changes in the diagnostic criteria except for newer recommendations of fever of at least 4 instead of 5 days duration. Recently, Echocardiography Criteria and Laboratory Criteria have been added to aid in the diagnosis of incomplete KD where all the historical diagnostic criteria are not present; this is now called the "incomplete form of KD" as opposed to "atypical form of KD". The word "atypical" is reserved for unusual presentations of KD such as those with hemophagocytic syndrome or nerve palsy. The treatment of KD includes infusion of high dose immunoglobulin. Patients non-responsive to immunoglobulin therapy are labeled as having "immunoglobulin resistant KD". The treatment of immunoglobulin resistant KD can be challenging and new therapies that have tried with some success. Late outcomes after 4 decades of treating these patients have recently been published. There has been some concern about increased risk for premature atherosclerosis in patients with childhood KD who had coronary artery abnormalities.

Kawasaki disease in children

“…Thus it seems likely that KD is associated with some widely distributed infectious agent 19. The coronavirus has been found in serological analysis of bodily secretions in up to 25% of cases of KD, although this is not felt to be the main causative factor 20 21. Work by Rowley et al has shown that oligoclonal IgA plasma cells infiltrate coronary arteries and other inflamed tissues in acute KD, suggestive of an antigen-driven response 22.…”

“…For those for whom this has not been effective, most clinicians would attempt a repeat dose of IVIg at 2 g/kg in order to achieve resolution of the inflammatory process 21 36 – 38. Thirteen per cent of a large series of patients with KD were found not to respond to initial IVIg treatment; however, only half of these were successfully treated with a second dose 38…”

Imaging studies in the diagnosis and management of vasculitis

“…Late US follow-up are based on the severity of the disease. When three or more supplemental US criteria (ie, perivascular brightness, Z-score of left descending or right coronary artery between 2 and 2.5, lack of vessel tapering, decreased left ventricular function, mitral regurgitation, and pericardial effusion) occur, diagnosis of KD can be made in absence of ectasia or aneurysm [33]. Baer et al [35] retrospectively analyzed charts of 100 patients with KD; 44% of patients displayed coronary artery ectasia or aneurysm on the initial US.…”

“…When a coronary artery is dilated without a segmental aneurysm, the vessel is considered ectatic. Coronary aneurysms are considered small if the are less than 5 mm, medium if between 5 and 8 mm, and giant if greater than 8 mm [33].…”

“…Thus, abdominal and subclavian arteriographies are recommended in patients with KD undergoing coronary angiography for the first time [34••]. Furthermore, X-ray angiography allows therapeutic options such as intraluminal thrombolytic therapy for a thrombus or percutaneous transluminal coronary angioplasty for stenosis [33].…”