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. 2018 Dec 14;9:2974. doi: 10.3389/fimmu.2018.02974

Table 1.

Table representing the principal hypotheses for the etiopathogenesis of KD.

Response to a pathogen Autoantibody or T cell driven autoimmune process Autoinflammatory process
Pros • Histological features suggesting an early innate immune response, followed by an adaptive immunity
• Plasmablast response such as in children with infectious diseases
• Intracytoplasmic inclusions and oligoclonal IgA antibodies
• Protective effect of breastfeeding
• Low rate of recurrence
• Seasonal variation and clusters of illness
• Virtual absence of KD in adulthood
• AECAs antibodies in acute KD (conflicting results) • Overlapping clinical features with AIDs
• Increased expression of genes related to the NLRP3 inflammasome, IL-1a and IL-1b, and caspase 1 in blood
• IL-1β and IL-1α implicated in myocarditis and aneurysm formation in LCWE -induced mouse model of KD
• Efficacy of IL-1 blockade
Cons • Unique gene expression pattern, differentiating KD from viral or bacterial diseases
• No evidence for person-to-person transmission
• Geospatial clustering at scales of 10-100 km
• Self-limited nature of KD
• Low rate of recurrence
• No concomitant autoimmune disease
• No familial aggregation with other autoimmune disease
• Selection of Fc-specific Treg cells in KD
• Polygenic nature of KD rather than a monogenic disease
• Monophasic course

For each hypothesis, data confirming or refuting are summarized. KD, Kawasaki disease; AIDs, autoinflammatory diseases; AECAs, anti-endothelial cell auto-antibodies; IL-1, interleukin 1; LCWE, Lactobacillus casei cell wall extract.