Tetsuhiro Kibata1, Yasuo Suzuki1, Shunji Hasegawa2, Takeshi Matsushige1, Takeshi Kusuda1, Madoka Hoshide1, Kazumasa Takahashi1, Seigo Okada1, Hiroyuki Wakiguchi1, Tadashi Moriwake3, Masashi Uchida4, Noriko Ohbuchi5, Takashi Iwai6, Masanari Hasegawa7, Kiyoshi Ichihara8, Mayumi Yashiro9, Nobuko Makino9, Yosikazu Nakamura9, Shouichi Ohga1. 1. Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. 2. Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan. Electronic address: shunji@yamaguchi-u.ac.jp. 3. Division of Pediatrics, National Hospital Organization Iwakuni Clinical Center, Iwakuni, Japan. 4. Division of Pediatrics, JCHO Tokuyama Central Hospital, Shunan, Japan. 5. Division of Pediatrics, Yamaguchi Red Cross Hospital, Yamaguchi, Japan. 6. Division of Pediatrics, Yamaguchi-ken Saiseikai Shimonoseki General Hospital, Shimonoseki, Japan. 7. Division of Pediatrics, Yamaguchi Grand Medical Center, Hofu, Japan. 8. Department of Laboratory Sciences, Faculty of Health Sciences, Yamaguchi University Graduate School of Medicine, Ube, Japan. 9. Department of Public Health, Jichi Medical University, Shimotsuke, Japan.
Abstract
BACKGROUNDS: Kawasaki disease (KD) is a systemic vasculitis of childhood involving coronary arteries. Treatment for intractable cases at a higher risk of cardiac sequelae remains controversial. METHODS: Clinical outcomes of KD patients diagnosed in Yamaguchi prefecture, Japan between 2003 and 2014 were analyzed using the medical records from all 14 hospitals covering the prefecture. The study included 1487 patients (male:female, 873:614; median age at diagnosis, 24months). RESULTS: The proportion of initial intravenous immunoglobulin (IVIG)-resistant patients increased from 7% to 23% during this decade, although no patients died. Twenty-four patients developed coronary artery lesions (CALs) over one month after the KD onset. The incidence of CAL in patients who received corticosteroid during the disease course (10/37; 27.0%) was higher than that in those who did not (14/1450; 0.97%, p=2.0×10(-35)). Nine patients who responded to initial IVIG plus corticosteroids had no CAL. Conversely, IVIG-resistant patients with alternate corticosteroid therapy more frequently developed CAL than those without it (10/28; 35.7% vs. 5/194; 2.6%, p=8.9×10(-10)). Multivariate analyses indicated corticosteroid therapy (p<0.0001), hyperbilirubinemia (p=0.0010), and a longer number of days before treatment (p=0.0005) as risk factors associated with CAL over a month after onset. The odds ratio of corticosteroid use increased from 18.3 to 43.5 if the cases were limited to initial IVIG non-responders and corticosteroid free-IVIG responders. CONCLUSIONS: IVIG-failure has recently increased. The incidence of CAL increased in intractable cases with prolonged corticosteroid use. Corticosteroid may not be alternate choice for IVIG-failure to reduce the risk of cardiac sequelae.
BACKGROUNDS: Kawasaki disease (KD) is a systemic vasculitis of childhood involving coronary arteries. Treatment for intractable cases at a higher risk of cardiac sequelae remains controversial. METHODS: Clinical outcomes of KDpatients diagnosed in Yamaguchi prefecture, Japan between 2003 and 2014 were analyzed using the medical records from all 14 hospitals covering the prefecture. The study included 1487 patients (male:female, 873:614; median age at diagnosis, 24months). RESULTS: The proportion of initial intravenous immunoglobulin (IVIG)-resistant patients increased from 7% to 23% during this decade, although no patients died. Twenty-four patients developed coronary artery lesions (CALs) over one month after the KD onset. The incidence of CAL in patients who received corticosteroid during the disease course (10/37; 27.0%) was higher than that in those who did not (14/1450; 0.97%, p=2.0×10(-35)). Nine patients who responded to initial IVIG plus corticosteroids had no CAL. Conversely, IVIG-resistant patients with alternate corticosteroid therapy more frequently developed CAL than those without it (10/28; 35.7% vs. 5/194; 2.6%, p=8.9×10(-10)). Multivariate analyses indicated corticosteroid therapy (p<0.0001), hyperbilirubinemia (p=0.0010), and a longer number of days before treatment (p=0.0005) as risk factors associated with CAL over a month after onset. The odds ratio of corticosteroid use increased from 18.3 to 43.5 if the cases were limited to initial IVIG non-responders and corticosteroid free-IVIG responders. CONCLUSIONS:IVIG-failure has recently increased. The incidence of CAL increased in intractable cases with prolonged corticosteroid use. Corticosteroid may not be alternate choice for IVIG-failure to reduce the risk of cardiac sequelae.
Authors: Rebecca A Porritt; Carol Chase Huizar; Edward J Dick; Shyamesh Kumar; Renee Escalona; Angela C Gomez; Stefani Marek-Iannucci; Magali Noval Rivas; Jean Patterson; Thomas G Forsthuber; Moshe Arditi; Mark Gorelik Journal: Front Immunol Date: 2021-04-09 Impact factor: 7.561