Background: Kawasaki disease (KD) is an acute inflammatory vasculitis that primarily affects children under 5 years of age. Kawasaki Disease Shock Syndrome (KDSS) is a rare complication characterized by hypotension and shock. Despite being the leading cause of heart disease in developed countries, diagnosing KD is challenging due to the lack of specific pathognomonic tests. Delayed treatment can increase the risk of long-term coronary artery abnormalities in up to 25% of patients. Case presentation: A 31-month-old boy initially presented with fever, lymphadenopathy, and pharyngeal congestion. The patient was treated with antibiotics due to positive Mycoplasma IgM and elevated inflammatory markers. Despite treatment, the patient a widespread rash, ulcers, lip peeling, and orbital swelling. The patient was transferred to the Pediatric Intensive Care Unit of a second hospital as a case of suspected septic shock. The patient received Intravenous immunoglobulin and corticosteroids without a diagnosis of KD. It was not until the 23rd day of illness that KD was diagnosed, and the absence of timely diagnosis resulted in aneurysmal dilation of all major coronary arteries with aneurysmal fusiform formation. Conclusion: The case highlights the importance of early recognition and diagnosis of KD and KDSS, recommending multidisciplinary evaluation. Clinicians should consider atypical KD in children who are unresponsive to antibiotics and have persistent fever. Positive infectious serology does not exclude the diagnosis of KD. In the absence of echocardiographic markers, early treatment should be prioritized to prevent long-term cardiac damage.
| Published in | American Journal of Pediatrics (Volume 11, Issue 4) |
| DOI | 10.11648/j.ajp.20251104.15 |
| Page(s) | 231-236 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2025. Published by Science Publishing Group |
Kawasaki Disease, Shock, Coronary Aneurysm, Acute Disease, Fever, Child
| [1] | McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association: 2023 update. Circulation. 2023; 147(16): e451-e483. |
| [2] | Games-Gonzalez LB, Moribe Quintero I, Cisneros-Castolo M, Varela Ortiz J, Munoz-Ramirez M, Garrido-Garcia M, Yamazaki-Nakashimada M. Kawasaki disease shock syndrome: unique and severe subtype of Kawasaki disease. Pediatrics International. 2018, 60, 781-790. |
| [3] | Zhao Z, Yuan Y, Gao L, Li Q, Wang Y, Zhao S. Predicting Kawasaki disease shock syndrome in children. Frontiers in Immunology. 2024; 15: 1400046. |
| [4] |
Xie X, Shi X, Liu M. The Roles of Genetic Factors in Kawasaki Disease: A Systematic Review and Meta-analysis of Genetic Association Studies. Pediatric Cardiology. 2018 Feb, 39(2): 207-225.
https://doi.org/10.1007/s00246-017-1760-0 Epub 2017 Nov 2. |
| [5] | Cheng M, Zheng G, Gao L, Zhang B. The relationship between Mycoplasma and Kawasaki disease in pediatric patients: an updated systematic review and meta-analysis. Archives of Rheumatology. 2023 Jun; 39(1): 140–148. |
| [6] | Friedman KG, Gauvreau K, Hamaoka-Okamoto A, Tang A, Berry E, Tremoulet AH, Mahavadi VS, Baker A, deFerranti SD, Fulton DR, et al. Coronary artery aneurysms in Kawasaki disease: risk factors for progressive disease and adverse cardiac events in the US population. Journal of the American Heart Association. 2016, 5, e003289. |
| [7] | Kim SH. Diagnosis of coronary artery abnormalities in Kawasaki disease: recent guidelines and z-score systems. Clinical and Experimental Pediatrics. 2022 Sep, 65(9), 430-438. |
| [8] | Kanegaye JT, et al. Recognition of a Kawasaki Disease Shock Syndrome. Pediatrics. 2009, 123(5), e783-e789. |
| [9] | Wang W, Jin Y, Cao J, Zhou S, Lin Z. Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome? Medicine (Baltimore). 2024; 103(33): e38167. |
| [10] | Nakip ÖS, Kesici S, Yüksekgönül AÜ, Bilginer Y, Özen S, Bayrakcı B. Kawasaki Disease Shock Syndrome: Think Earlier, Treat Intensively. J Pediatr Emerg Intensive Care Med. 2023; 10(3): 216-220. |
| [11] | Zhang M, Wang C, Li Q, Wang H, Li X. Risk factors and an early predictive model for Kawasaki disease shock syndrome in Chinese children. Italian Journal of Pediatrics. 2024 Feb 3; 50(1): 22. |
| [12] | Li B, Liu X, Shao S, Wu P, Wu M, Liu L, Hua Y, Duan H, Zhou K, Wang C. Predictive value of coagulation profiles for Kawasaki disease shock syndrome: a prospective cohort study. Frontiers in Pediatrics. 2024; 12: 1450710. |
| [13] | Miyata K, Bainto EV, Sun X, Jain S, Dummer KB, Burns JC, Tremoulet AH. Infliximab for intensification of primary therapy for patients with Kawasaki disease and coronary artery aneurysms at diagnosis. Archives of Disease in Childhood. 2023, 108, 833-838. |
| [14] | Ae R, Abrams JY, Maddox RA, Schonberger LB, Nakamura Y, Kuwabara M, Makino N, Matsubara Y, Kosami K, Sasahara T, et al. Corticosteroids Added to the Initial Intravenous Immunoglobulin Treatment for the Prevention of Coronary Artery Abnormalities in High-Risk patients with Kawasaki Disease. Journal of the American Heart Association. 2020, 9, e015308. |
| [15] | Liu ZJ, Hsu WF. Refractory Kawasaki disease with a giant aneurysm successfully treated with infliximab and enoxaparin: a case report. Journal of Pediatric Health Care. 2025, 39(3), 459-465. |
| [16] | Li Z, Zhang Z, Tang Y, et al. The therapeutic window of intravenous immunoglobulin in Kawasaki disease: correlation with coronary artery lesions and cardiac sequelae. Italian Journal of Pediatrics. 2023; 49(1): 51. |
APA Style
Sulaiman, M., Ghazy, H., Sulaiman, Y., Sulaiman, A., Gendi, S. (2025). A Missed Kawasaki Disease Shock Syndrome Diagnosis Resulting in Coronary Complications. American Journal of Pediatrics, 11(4), 231-236. https://doi.org/10.11648/j.ajp.20251104.15
ACS Style
Sulaiman, M.; Ghazy, H.; Sulaiman, Y.; Sulaiman, A.; Gendi, S. A Missed Kawasaki Disease Shock Syndrome Diagnosis Resulting in Coronary Complications. Am. J. Pediatr. 2025, 11(4), 231-236. doi: 10.11648/j.ajp.20251104.15
Share This Article
Twitter
Linked In
Facebook
Copy
Download@article{10.11648/j.ajp.20251104.15,
author = {Milad Sulaiman and Heba Ghazy and Yara Sulaiman and Alena Sulaiman and Salwa Gendi},
title = {A Missed Kawasaki Disease Shock Syndrome Diagnosis Resulting in Coronary Complications},
journal = {American Journal of Pediatrics},
volume = {11},
number = {4},
pages = {231-236},
doi = {10.11648/j.ajp.20251104.15},
url = {https://doi.org/10.11648/j.ajp.20251104.15},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20251104.15},
abstract = {Background: Kawasaki disease (KD) is an acute inflammatory vasculitis that primarily affects children under 5 years of age. Kawasaki Disease Shock Syndrome (KDSS) is a rare complication characterized by hypotension and shock. Despite being the leading cause of heart disease in developed countries, diagnosing KD is challenging due to the lack of specific pathognomonic tests. Delayed treatment can increase the risk of long-term coronary artery abnormalities in up to 25% of patients. Case presentation: A 31-month-old boy initially presented with fever, lymphadenopathy, and pharyngeal congestion. The patient was treated with antibiotics due to positive Mycoplasma IgM and elevated inflammatory markers. Despite treatment, the patient a widespread rash, ulcers, lip peeling, and orbital swelling. The patient was transferred to the Pediatric Intensive Care Unit of a second hospital as a case of suspected septic shock. The patient received Intravenous immunoglobulin and corticosteroids without a diagnosis of KD. It was not until the 23rd day of illness that KD was diagnosed, and the absence of timely diagnosis resulted in aneurysmal dilation of all major coronary arteries with aneurysmal fusiform formation. Conclusion: The case highlights the importance of early recognition and diagnosis of KD and KDSS, recommending multidisciplinary evaluation. Clinicians should consider atypical KD in children who are unresponsive to antibiotics and have persistent fever. Positive infectious serology does not exclude the diagnosis of KD. In the absence of echocardiographic markers, early treatment should be prioritized to prevent long-term cardiac damage.},
year = {2025}
}
TY - JOUR T1 - A Missed Kawasaki Disease Shock Syndrome Diagnosis Resulting in Coronary Complications AU - Milad Sulaiman AU - Heba Ghazy AU - Yara Sulaiman AU - Alena Sulaiman AU - Salwa Gendi Y1 - 2025/11/28 PY - 2025 N1 - https://doi.org/10.11648/j.ajp.20251104.15 DO - 10.11648/j.ajp.20251104.15 T2 - American Journal of Pediatrics JF - American Journal of Pediatrics JO - American Journal of Pediatrics SP - 231 EP - 236 PB - Science Publishing Group SN - 2472-0909 UR - https://doi.org/10.11648/j.ajp.20251104.15 AB - Background: Kawasaki disease (KD) is an acute inflammatory vasculitis that primarily affects children under 5 years of age. Kawasaki Disease Shock Syndrome (KDSS) is a rare complication characterized by hypotension and shock. Despite being the leading cause of heart disease in developed countries, diagnosing KD is challenging due to the lack of specific pathognomonic tests. Delayed treatment can increase the risk of long-term coronary artery abnormalities in up to 25% of patients. Case presentation: A 31-month-old boy initially presented with fever, lymphadenopathy, and pharyngeal congestion. The patient was treated with antibiotics due to positive Mycoplasma IgM and elevated inflammatory markers. Despite treatment, the patient a widespread rash, ulcers, lip peeling, and orbital swelling. The patient was transferred to the Pediatric Intensive Care Unit of a second hospital as a case of suspected septic shock. The patient received Intravenous immunoglobulin and corticosteroids without a diagnosis of KD. It was not until the 23rd day of illness that KD was diagnosed, and the absence of timely diagnosis resulted in aneurysmal dilation of all major coronary arteries with aneurysmal fusiform formation. Conclusion: The case highlights the importance of early recognition and diagnosis of KD and KDSS, recommending multidisciplinary evaluation. Clinicians should consider atypical KD in children who are unresponsive to antibiotics and have persistent fever. Positive infectious serology does not exclude the diagnosis of KD. In the absence of echocardiographic markers, early treatment should be prioritized to prevent long-term cardiac damage. VL - 11 IS - 4 ER -
Department of Pediatrics, Kidsheart Medical Center, Dubai, United Arab Emirates
Department of Pediatrics, Kidsheart Medical Center, Dubai, United Arab Emirates
Department of Pediatrics, Kidsheart Medical Center, Dubai, United Arab Emirates
Department of Pediatrics, Kidsheart Medical Center, Dubai, United Arab Emirates
Department of Pediatrics, Kidsheart Medical Center, Dubai, United Arab Emirates
