Overlapping Features in Kawasaki Disease-Related Arthritis and Systemic-Onset Juvenile Idiopathic Arthritis: A Nationwide Study in Japan

Hikaru Kanemasa, Etsuro Nanishi, Hidetoshi Takada, Masataka Ishimura, Hisanori Nishio, Satoshi Honjo, Hiroshi Masuda, Noriko Nagai, Takahiro Nishihara, Tohru Ishii, Takenori Adachi, Satoshi Hara, Lisheng Lin, Yoshie Tomita, Junji Kamizono, Osamu Komiyama, Urara Kohdera, Saori Tanabe, Atsuo Sato, Shinya HidaMayumi Yashiro, Nobuko Makino, Yosikazu Nakamura, Toshiro Hara, Shouichi Ohga

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7 Citations (Scopus)


Background: Arthritis may occur after the diagnosis of Kawasaki disease (KD). Most cases are self-limiting; however, some patients require prolonged treatment. Method: To characterize KD-related arthritis, 14 patients who required arthritis treatment within 30 days after the diagnosis of KD were recruited from the 23rd KD survey in Japan. Twenty-six additional patients were included from our tertiary center and literature review cohorts. Results: The estimated prevalence of KD-related arthritis in Japan was 48 per 100,000 KD patients. Patients with KD-related arthritis had an older age at onset (52 vs. 28 months, P = 0.002) and higher rate of intravenous immunoglobulin (IVIG) resistance in comparison to those without arthritis (86 vs. 17%, P < 0.001). Among 40 patients, 18 had arthritis in the acute phase KD (continued fever-onset type) and 22 did in the convalescent phase (interval fever-onset type). Both showed a similar rate of complete KD or IVIG response. Interval-type patients required biologics for arthritis control less frequently (5 vs. 39%, P = 0.02) and had a higher 2-year off-treatment rate (100 vs. 43%, P = 0.009) than continued-type ones. Interval-types showed lower serum ferritin and interleukin-18 levels than continued-types. When continued-types were grouped according to whether or not they required biologics (n = 7 and n = 11, respectively), the former subgroup had higher ferritin and interleukin-18 levels (P = 0.01 and 0.02, respectively). A canonical discriminant analysis differentiated interval-type from continued-type with the combination of age, time to arthritis, and the ferritin and matrix metalloproteinase-3 levels. Conclusion: Arthritis requiring treatment is a rare complication of KD. KD-associated arthritis includes interval-type (KD-reactive) and continued-type (true systemic-onset juvenile idiopathic arthritis [JIA] requiring biologics), and overlapping arthritis, suggesting the pathophysiological continuity of autoinflammation between KD and JIA.

Original languageEnglish
Article number597458
JournalFrontiers in Pediatrics
Publication statusPublished - Jul 20 2021

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health


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