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Kawasaki Disease

Kawasaki Disease: The Old and the New
from Infections in Medicine ®
Benjamin Estrada, MD

Kawasaki disease (KD) is a form of vasculitis that is frequently associated with the development of coronary aneurysms. It is also one of the most common causes of acquired heart disease in children. Although no causative agent has been identified, the epidemiologic and clinical characteristics suggest an infectious cause. Treatment of KD consists of the administration of intravenous immunoglobulin (IVIG) and aspirin given at high doses. This therapeutic approach has been shown to be effective in the prevention of coronary aneurysm development. The following is a summary of recent advances in the pathogenesis, epidemiology, and treatment of KD.

Rowley and collaborators[1] recently developed a study that has added to the advances toward identifying a causative agent for KD. This study demonstrated the presence of IgA plasma cell infiltration in the pancreas, kidneys, and coronary arteries of patients who died of KD. In addition, significant concentrations of IgA plasma cells were found in the proximal respiratory tract, which suggests a respiratory port of entry for the KD agent. The investigators state that children with KD frequently have a preceding episode of upper respiratory tract infection. Although histologic findings similar to the ones reported in this study have not yet been reported in any other human disease process, plasma cell infiltration is observed in other mammal infections, such as those caused by the equine arteritis virus and Aleutian mink disease virus.

One of the most significant challenges for the clinician is the diagnosis of KD in those children who do not present with all the typical manifestations. In a recent study, Barone and collaborators[2] suggest that performing rapid tests for adenovirus may be helpful in differentiating adenoviral infection from the clinically similar atypical KD. Using this diagnostic tool might help avoid administration of IVIG to children with adenoviral disease.[2]

It is well known that IVIG is effective in the prevention of coronary aneurysms in KD. However, in approximately 10% of patients who receive this product, coronary artery disease eventually develops. Mori and collaborators[3] have shown that persistently elevated concentrations of C-reactive protein and leukocytosis after treatment with IVIG were predictors for development of coronary artery lesions. Other investigators in Japan also have demonstrated that C-reactive protein levels greater than 10 mg/dL, lactate dehydrogenase levels greater than 590 IU/L, and hemoglobin levels greater than 10 g/dL at admission were considered predictors of nonresponsiveness to high-dose IVIG therapy in patients with KD. Nonresponsiveness to IVIG treatment in this study was defined as the persistence of fever for more than 5 days after its administration. It has been suggested that children who are at risk for nonresponsiveness could be identified early based on these laboratory parameters, and additional therapy could be considered during the early stage of the disease process.[4]

For those patients who have long-term sequelae from coronary artery disease secondary to KD, additional management has recently included catheter interventional treatment. This interventional modality has been performed with relative success, as presented in a recent report from the Japanese Pediatric Interventional Cardiology Investigation Group.[5] The procedures used in this approach include percutaneous transluminal coronary angioplasty, percutaneous transluminal coronary rotational ablation, directional coronary atherectomy, and stent implantation. Although these interventions appear promising, their success is highly dependent on the availability of experienced interventional cardiologists. In addition, there are no specific guidelines to determine patient candidates at this time.

It is clear that mortality and other sequelae of KD have significantly decreased with the use of IVIG. However, more research is needed to identify the cause of this disorder, assess the prognosis before and after medical treatment, and evaluate the safety of invasive therapeutic approaches when significant sequelae have developed.

Dr. Estrada is assistant professor of pediatrics, division of pediatric infectious diseases, University of South Alabama, Mobile.