Abstract
The etiology of Kawasaki disease (KD), the leading cause of acquired heart disease in children, is currently unknown. Epidemiology supports a relationship of KD to an infectious disease. Several pathological mechanisms are being considered, including a superantigen response, direct invasion by an infectious etiology or an autoimmune phenomenon. Treating affected patients with intravenous immunoglobulin is effective at reducing the rates of coronary aneurysms. However, the role of B cells and antibodies in KD pathogenesis remains unclear. Murine models are not clear on the role for B cells and antibodies in pathogenesis. Studies on rare aneurysm specimens reveal plasma cell infiltrates. Antibodies generated from these aneurysmal plasma cell infiltrates showed cross-reaction to intracellular inclusions in the bronchial epithelium of a number of pathologic specimens from children with KD. These antibodies have not defined an etiology. Notably, a number of autoantibody responses have been reported in children with KD. Recent studies show acute B cell responses are similar in children with KD compared to children with infections, lending further support of an infectious disease cause of KD. Here, we will review and discuss the inconsistencies in the literature in relation to B cell responses, specific antibodies, and a potential role for humoral immunity in KD pathogenesis or diagnosis.
Highlights
Kawasaki disease (KD), known as Kawasaki syndrome, is the leading cause of acquired cardiac disease in children [1]
Success with anti-TNF monoclonal antibodies seemingly argues against a significant role of B cells, as this would effectively release a suppressive action of TNF on B cell proliferation
Single dose of intravenous immunoglobulin (IVIG) is better than splitting doses [68]
Summary
Kawasaki disease (KD), known as Kawasaki syndrome, is the leading cause of acquired cardiac disease in children [1]. Roughly one-quarter of the children meeting clinical criteria will go on to have coronary artery inflammation, including aneurysms. Incomplete cases, those which do not fulfill four of five of the classic criteria, have similar risk of coronary aneurysms [6]. To add to the diagnostic confusion, several infectious etiologies have been independently associated with aneurysms [15]. It remains a frustrating diagnosis because of the unknown etiology, clinical variability, lack of specific testing, and unclear pathogenesis
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