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Incidence of chronic immune-mediated inflammatory diseases after diagnosis with Kawasaki disease: a population-based cohort study

Fung SG, Webster R, Kuenzig ME, Knight B, Batthish M, Robinson C, Chanchlani R, Benchimol EI, Jimenez-Rivera C. Rheumatology (Oxford). 2021; Sep 8 [Epub ahead of print]. DOI:

Objectives — Kawasaki disease (kDa) is an immune-mediated vasculitis of childhood with multi-organ inflammation. We determined the risk of subsequent immune-mediated inflammatory disease (IMID), including arthritis, type 1 diabetes, inflammatory bowel disease (IBD), autoimmune liver disease (ALD), primary sclerosing cholangitis (PSC) and multiple sclerosis (MS).

Methods — We conducted a matched population-based cohort study using health administrative data from Ontario, Canada. Children aged <18 years born between 1991-2016 diagnosed with kDa (n = 3,753) were matched to 5 non-kDa controls from the general population (n = 18 749). We determined the incidence of IMIDs after resolution of kDa. Three- and 12-month washout periods were used to exclude kDa-related symptoms.

Results —There was an elevated risk of arthritis in kDa patients compared with non-kDa controls starting three months after index date (103.0 vs 12.7 per 100 000 person-years (PYs); incidence rate ratio (IRR) 8.07 (95% CI 4.95-13.2); hazard ratio (HR) 8.08 (95% CI 4.95-13.2), resulting in the overall incidence of IMIDs being elevated in kDa patients (175.1 vs 68.0 per 100 000 PYs; IRR 2.58 (95% CI 1.93-3.43); HR 2.58, 95% CI 1.94-3.43). However, there was no increased risk for diabetes, IBD, ALD, PSC or MS in kDa patients. Similar results were observed using a 12-month washout period.

Conclusions — Children diagnosed with kDa were at increased risk of arthritis following the acute kDa event, but not other IMIDs. Health care providers should monitor for arthritis in children following a diagnosis of kDa.