Pediatric Infectious Disease

Register      Login

VOLUME 4 , ISSUE 3 ( July-September, 2022 ) > List of Articles


Retrospective Cohort Study of Clinical Profile and IVIg Resistance in Children with Incomplete Kawasaki Disease in a Tertiary Care Center

Bhaskar Shenoy, Basavanhalli Chandregowda Arun

Keywords : Coronary artery abnormality, Incomplete Kawasaki disease, Infliximab, Intravenous immunoglobulin

Citation Information : Shenoy B, Arun BC. Retrospective Cohort Study of Clinical Profile and IVIg Resistance in Children with Incomplete Kawasaki Disease in a Tertiary Care Center. Pediatr Inf Dis 2022; 4 (3):83-85.

DOI: 10.5005/jp-journals-10081-1281

License: CC BY-NC 4.0

Published Online: 31-08-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Background: Diagnosis of incomplete Kawasaki disease (KD) is a clinical challenge in the absence of a specific diagnostic test. Diagnosis of KD is based mainly on the typical constellation of clinical signs and symptoms. Incomplete KD is much more difficult to diagnose because they present with fewer clinical features. Hence, diagnosis and treatment are often delayed in such cases and the response to treatment with intravenous immunoglobulin (IVIg) is poor. A high index of suspicion is necessary to diagnose and manage the children presenting as incomplete KD. Objectives: To study the clinical spectrum of incomplete KD and to assess the response to IVIg therapy. Materials and methods: A retrospective cohort study of children with incomplete KD at a tertiary care hospital from January 2010 to April 2018. Diagnosis and treatment were based on American Heart Association (AHA) guidelines. Results: Fifty-four out of 94 KD cases were incomplete KD (57.4%). Most of the incomplete KD cases were in infants (48.1%). The mean duration of fever at diagnosis was 6.3 days in complete KD and 6.9 days in incomplete KD. Clinical manifestations were oral mucosal changes (79.6%), conjunctival injection (68.5%), and polymorphous exanthema (64.8%). Less common clinical manifestations were extremity changes (14.8%), cervical lymphadenopathy (33.3%), irritability (53.7%), diarrhea (31.4%), vomiting (20.3%), and Bacillus Calmette-Guerin (BCG) scar flare-up (11.1%). Coronary artery abnormality (CAA) was detected in 27 cases (50%). Forty-nine out of 54 cases showed clinical resolution to IVIg (90.7%), 5 were resistant to the first dose of IVIg, 4 responded to the second dose of IVIg, and 1 required infliximab. Conclusion: Incomplete KD is more often associated with CAA. Clinicians should have a high index of suspicion when fever persists for 5 days or more and is associated with any of the principal clinical manifestations. Few laboratory tests and echocardiography may assist in the early diagnosis and treatment. The majority of the children responded to IVIg.

PDF Share
  1. McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation 2017;135(17):e927–e999. DOI: 10.1161/CIR.0000000000000484.
  2. Manlhiot C, Christie E, McCrindle BW, et al. Complete and incomplete Kawasaki disease: two sides of the same coin. Eur J Pediatr 2012;171(4):657–662. DOI: 10.1007/s00431-011-1631-2.
  3. Sudo D, Monobe Y, Yashiro M, et al. Coronary artery lesions of incomplete Kawasaki disease: a nationwide survey in Japan. Eur J Pediatr 2012;171(4):651–656. DOI: 10.1007/s00431-011-1630-3.
  4. Gorczyca D, Postępski J, Olesińska E, et al. The clinical profile of Kawasaki disease of children from three polish centers: a retrospective study. Rheumatol Int 2014;34(6):875–880. DOI: 10.1007/s00296-013-2836-7.
  5. Yu JJ. Diagnosis of incomplete Kawasaki disease. Korean J Pediatr 2012;55(3):83. DOI: 10.3345/kjp.2012.55.3.83.
  6. Perrin L, Letierce A, Guitton C, et al. Comparative study of complete vs incomplete Kawasaki disease in 59 pediatric patients. Joint Bone Spine 2009;76(5):481–485. DOI: 10.1016/j.jbspin.2008.11.015.
  7. Shivalingam G, Prashanth GP, Hebbal K, et al. Clinical presentation and cardiovascular outcome in complete vs incomplete Kawasaki disease. Indian Pediatr 2017;54(10):844–847. DOI: 10.1007/s13312-017-1147-6.
  8. Balasubramanian S, Krishna MR, Dhanalakshmi K, et al. Factors associated with delay in diagnosis of Kawasaki disease in india. Indian Pediatr 2012;49(8):663–665. DOI: 10.1007/s13312-012-0130-5.
  9. Baer AZ, Rubin LG, Shapiro CA, et al. Prevalence of coronary artery lesions on the initial echocardiogram in Kawasaki syndrome. Arch Pediatr Adolesc Med 2006;160(7):686–690. DOI: 10.1001/archpedi.160.7.686.
  10. Mori M, Miyamae T, Imagawa T, et al. Meta-analysis of the results of intravenous gamma globulin treatment of coronary artery lesions in Kawasaki disease. Mod Rheumatol 2004;14(5):361–366. DOI: 10.3109/s10165-004-0324-3.
  11. Oates-Whitehead RM, Baumer JH, Haines L, et al. Intravenous immunoglobulin for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev 2003;2003(4):CD004000. DOI: 10.1002/14651858.CD004000.
  12. Tremoulet AH, Best BM, Song S, et al. Resistance to intravenous immunoglobulin in children with Kawasaki disease. J Pediatr 2008;153(1):117–121. DOI: 10.1016/j.jpeds.2007.12.021.
  13. Durongpisitkul K, Soongswang J, Laohaprasitiporn D, et al. Immunoglobulin failure and retreatment in Kawasaki disease. Pediatr Cardiol 2003;24(2):145–148. DOI: 10.1007/s00246-002-0216-2.
  14. Özdemir H, Çiftçi E, Tapısız A, et al. Clinical and epidemiological characteristics of children with Kawasaki disease in turkey. J Trop Pediatr 2010;56(4):260–262. DOI: 10.1093/tropej/fmp110.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.