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Volume 4, Issue 1

January 2024

Etiology, Pathophysiology, Diagnosis and Management of Kawasaki Disease

Abdulghani Alzamzami, Osama Almuqaytib, Ahmad Alsaadi, Ahmed Alasmari, Khalid Alsuwat, Ahmed Al Abdullah, Abdulaziz Alomair, Asalah Alomair, Noor Alawami, Anas Alamodi, Abdullah Alismail,Bandar Alenezi

DOI: http://dx.doi.org/10.52533/JOHS.2024.40109

Keywords: Kawasaki Disease, Pediatric Vasculitis, Immune Dysregulation, Coronary Artery Abnormalities, Intravenous Immunoglobulin

Kawasaki Disease (KD) has been a challenge in medicine ever since it was first described in 1967. This illness presents as a fever accompanied by a range of mucocutaneous symptoms making it crucial to have sharp diagnostic skills. It is characterized by inflammation of arteries throughout the body particularly affecting the cardiovascular system. Suspected triggers include agents, genetic factors and immune responses. KD pathophysiology is closely linked to dysregulation, especially involving inflammatory cytokines. Diagnosis relies on criteria such as fever for more than five days and specific symptoms. The primary pharmacological treatments involve immunoglobulin (IVIG) and aspirin to reduce inflammation and prevent complications in the arteries. In cases corticosteroids and immunomodulatory agents may be considered. Long term follow-up with collaboration among healthcare professionals is essential for monitoring any abnormalities in the arteries. Echocardiography plays a role in this process by providing insights into complications that may arise over time. Ongoing research aims to explore options as part of a comprehensive approach to KD management that emphasizes continuous research and an adaptable strategy for achieving the best possible outcomes.


Kawasaki Disease (KD) poses a challenge in the field of medicine ever since it was first described by Tomisaku Kawasaki back in 1967. It mainly affects children and manifests as an illness, with an unknown origin, complex underlying mechanisms and diagnostic requirements that demand keen observation skills. Over the years extensive research has been conducted to unravel the mysteries surrounding this condition and improve treatment approaches. Despite these efforts the exact cause of KD continues to remain a puzzle (1, 2). Some proposed triggers include agents, genetic predisposition and immunological factors. Recent studies highlight the role of acquired immune responses in the development of disease. Kawasaki Disease (KD) is characterized by inflammation of arteries throughout the body particularly affecting the cardiovascular system (3, 4). This inflammation can lead to complications in some patients concerning issues with arteries. Understanding how this disease works is crucial for management. Current evidence suggests that an abnormal immune response, possibly triggered by an agent in individuals with genetic susceptibility initiates widespread inflammation and arterial inflammation (5, 6). The activation of both acquired pathways causes the release of pro inflammatory molecules like tumor necrosis factor alpha (TNF α) and interleukin 6 (IL 6). These inflammatory reactions contribute to symptoms seen in KD, such as fever, skin manifestations and systemic involvement (7, 8). Diagnosing KD can be challenging since there are no biomarkers instead it relies on clinical criteria. The guidelines provided by the American Heart Association (AHA) emphasize prolonged fever lasting than five days along with clinical features like conjunctivitis, mouth sores, rashes, changes in limbs appearance and swelling of lymph nodes in the neck. Additional tests like increased levels of acute phase reactants and echocardiography to assess artery involvement can help confirm the diagnosis. However, since there isn't a test for KD yet clinical expertise plays a vital role in managing suspected cases. The focus of KD management revolves around reducing inflammation preventing complications, in the arteries and providing care (9, 10). The primary treatment for this involves administering immunoglobulin (IVIG) in combination with aspirin. This approach aims to lessen the response and lower the chances of developing artery aneurysms. High dose aspirin is initially prescribed during the phase. Then it is switched to a lower dose, for its antiplatelet effects in the recovery phase. In cases where coronary artery complications persist or if the condition does not respond well corticosteroids may be considered. Managing Kawasaki Disease goes beyond the phase. It involves long term follow up and monitoring for any abnormalities in the coronary arteries. Echocardiography plays a role in assessing the size of these arteries and guiding treatment (11, 12). Collaboration between pediatricians, cardiologists and rheumatologists is vital to ensure care due to the nature of this disease. Therefore, Kawasaki Disease presents a challenge in medicine because its cause is not fully understood its underlying mechanisms are intricate and accurate diagnosis requires keen diagnostic skills (13). The evolving understanding of dysregulation in Kawasaki Disease informs treatment strategies which involve administering IVIG (intravenous immunoglobulin) using aspirin therapy and closely monitoring for potential complications affecting the coronary arteries (14). While diagnosis still relies on criteria, ongoing research holds promise for uncovering factors and improving therapeutic approaches for managing this fascinating and clinically significant condition, among children. This study aims to provide an overview of the causes, mechanisms, diagnosis and management of Kawasaki Disease.


Kawasaki Disease (KD) displays a range of symptoms that can be challenging for healthcare providers to diagnose. The initial phase is characterized by a prolonged fever, a clue for doctors to consider KD a possibility. The involvement of the system adds complexity to the picture with symptoms, like "strawberry tongue," non-purulent conjunctivitis in both eyes and a rash with various patterns. Changes in the extremities and swollen lymph nodes in the neck also contribute to the presentation. The systemic impact of KD is reflected in irritability, fatigue, and gastrointestinal symptoms, showing how the immune response affects the body. However, KD can evolve over time and not all classic features appear simultaneously. This complexity emphasizes the need for recognition to prevent complications in the arteries (15). Managing KD involves approaches starting with recognition and continuing with long-term monitoring. Intravenous immunoglobulin (IVIG) and aspirin are treatments that reduce inflammation and prevent blood clots (16). Corticosteroids and immunomodulatory drugs may be considered if cases are resistant to treatment. Long-term follow-ups by healthcare professionals working together are crucial for monitoring any abnormalities in the arteries. Echocardiography plays a role in the detection of evolving complications. Ongoing studies are investigating ways to treat KD, which shows how the management of this condition is constantly changing.

Clinical Manifestation

Kawasaki Disease (KD) is a condition that mainly affects children. It presents stages characterized by both mucocutaneous symptoms. The disease begins with a phase where the child experiences a persistent fever that doesn't respond well to fever reducing medications, which then leads to other manifestations. This fever phase is an indicator requiring careful observation, by healthcare professionals. One notable feature of KD is the appearance of a "strawberry tongue," along with redness and cracking of the lips. Additionally, there is purulent conjunctivitis, which highlights the systemic nature of the disease. The integumentary system also plays a role in KD as it develops a rash characterized by red and raised lesions that can be seen on the trunk, limbs and perineal area. It's crucial for clinicians to have an understanding of these skin manifestations for recognition and appropriate intervention. Changes in the extremities are another aspect of KDs presentation. Swelling and redness accompanied by peeling contribute to a pattern resembling gloves and socks (17, 18). These peripheral changes do not serve as features but also indicate the impact of the disease on vascular integrity. Understanding these physical changes is crucial to have an assessment and appropriate management. Cervical lymphadenopathy is a feature of KD, frequently occurring on one side and causing tenderness during examination. The presence of lymph nodes adds complexity to the picture making it more challenging for healthcare providers to diagnose. Identifying this lymphadenopathy is essential for an understanding of KD. Helps differentiate it from other similar pediatric illnesses. KD affects the body, not specific areas causing various symptoms that impact the overall well-being of affected children. Signs like irritability, tiredness and decreased appetite become apparent reflecting the consequences of a response gone awry (19, 20). Gastrointestinal issues such as stomach pain, diarrhea and vomiting further emphasize that KD affects the system. The changing clinical presentation of KD highlights how crucial it is for healthcare providers to recognize it promptly. However, it's important to note that the classic features may only sometimes be present initially, making diagnosis challenging. Clinicians must maintain a level of suspicion due to KDs evolving nature and its potential to mimic febrile illnesses in children. The systemic inflammation caused by KD requires intervention to reduce the risk of complications in arteries—a significant concern associated with this disease. Understanding the range of symptoms and their dynamic progression is vital in ensuring that children, with KD receive prompt and appropriate care. As we continue to learn more about KD, ongoing research plays a role, in improving criteria and enhancing therapeutic strategies. This highlights the importance of managing KD in a clinical setting.


The management of Kawasaki Disease (KD), in a setting requires a comprehensive approach to address the various challenges presented by this inflammatory condition. A key aspect of management is recognition as it plays a crucial role in minimizing the risk of complications related to coronary artery abnormalities that are often associated with KD. The diagnostic criteria provided by the American Heart Association (AHA) highlight the importance of factors including fever for more than five days and specific clinical features such as conjunctivitis, mucositis, rash, changes in extremities and cervical lymphadenopathy (21, 22). It is important to note that not all criteria may appear simultaneously underscoring the significance of expertise when suspecting cases of KD. Once diagnosed the primary focus shifts towards reducing inflammation. Intravenous immunoglobulin (IVIG) administered at a dosage (2g/kg) plays a role in KD treatment. Its timely initiation within ten days of illness onset has demonstrated effectiveness in decreasing the risk of artery abnormalities. Although the exact mechanism by which IVIG works in KD is not fully understood it is believed to regulate response and exert inflammatory effects. Administering IVIG promptly has been associated with higher treatment success rates. Additionally, aspirin therapy plays a role, in managing KD alongside treatment. During the phase dose aspirin is prescribed to treat inflammation and reduce fever. As the patient progresses towards a fever state within 48-72 hours, after receiving IVIG treatment the dosage of aspirin is gradually lowered to take advantage of its antiplatelet properties. This approach does not help reduce inflammation. Also aids in preventing blood clot formation, in the coronary arteries. In cases where complications in the arteries persist or become difficult to manage doctors may consider using corticosteroids. However, it is important to evaluate the risks and benefits associated with their use. In some instances, where patients do not respond well to immunoglobulin (IVIG) alternative treatments like infliximab, which's an immunomodulatory agent, may be explored. Managing Kawasaki Disease goes beyond the phase and emphasizes the need for long-term follow-up and diligent monitoring of any abnormalities in the coronary arteries. Echocardiography plays a role in this aspect by providing information about the size of the coronary arteries and guiding ongoing treatment decisions. Regular echocardiograms are conducted during stages of treatment. During the phase at two weeks six weeks and subsequently at regular intervals. Help detect any developing abnormalities in the coronary arteries at an early stage. Given its complexity managing Kawasaki Disease requires an approach involving healthcare professionals such as pediatricians, cardiologists and rheumatologists who work together to develop a well-informed treatment strategy. Regular follow-up visits are essential for assessing progress, addressing any emerging concerns that may arise and adjusting management strategies as necessary. The importance of management and surveillance cannot be overstated considering that Kawasaki Disease can have long term effects on the coronary arteries. If abnormalities continue or worsen doctors may consider using treatments, like anticoagulation or antiplatelet therapy to reduce risks. As research on KD advances scientists are exploring innovative ways to treat the condition. Recent studies have looked into the advantages of statins, TNF agents and other therapies that modulate the immune system in certain situations. However, it is important to note that the evidence supporting these interventions is still in the stages of development and their regular use in managing Kawasaki Disease is not yet firmly established. In summary, effectively managing Kawasaki Disease requires an adaptable approach that begins with recognition and extends to careful long term follow up. The combination of immunoglobulin, aspirin therapy and cautious use of corticosteroids forms the core of treatments aimed at reducing inflammation and preventing complications in the coronary arteries. Ongoing monitoring through echocardiography plays a role in detecting any emerging abnormalities in the coronary arteries in a timely manner. Collaborative efforts among healthcare professionals and continued exploration of strategies through research underscore the need for a comprehensive and evolving approach to optimize outcomes, for individuals affected by Kawasaki Disease.


Kawasaki Disease poses a challenge in the field of medicine due to its mysterious origins and complex physiological processes. The varied symptoms observed require abilities. Current management strategies rely on an understanding of immune system dysregulation. Involve the use of IVIG, aspirin and careful monitoring. The absence of biomarkers highlights the role that clinical expertise plays in making accurate diagnoses. The multifaceted management approach combines medication interventions with long term observation reflecting the nature of KD. Collaboration among healthcare professionals is vital to develop a strategy that emphasizes research to improve diagnostic criteria and treatment methods. As we continue to learn more about managing KD it remains essential to adopt an adaptable approach for outcomes, in affected individuals.


Conflict of interest

There is no conflict of interest


No funding

Ethical consideration

Non applicable

Data availability

Data that support the findings of this study are embedded within the manuscript.

Author contribution

All authors contributed to conceptualizing, data drafting, collection and final writing of the manuscript.