Short answer · Medically reviewed summary · Last updated: 2026-05-08
The ICD-10 code for Kawasaki Disease is M30.3, while the older ICD-9-CM classification system designated the condition as 446.1. These diagnostic codes are essential for medical billing, insurance reimbursement, and tracking the clinical documentation of Kawasaki Disease within healthcare systems globally. What is the clinical significance of Kawasaki Disease? Kawasaki Disease is an acute, systemic vasculitis that primarily affects children, characterized by inflammation of the medium-sized arteries, particularly the coronary arteries.
ICD10 code of Kawasaki Disease and ICD9 code
ICD-10 and ICD-9 codes for Kawasaki Disease, with classification details for clinicians, coders and patients.
The ICD-10 code for Kawasaki Disease is M30.3, while the older ICD-9-CM classification system designated the condition as 446.1. These diagnostic codes are essential for medical billing, insurance reimbursement, and tracking the clinical documentation of Kawasaki Disease within healthcare systems globally.
What is the clinical significance of Kawasaki Disease?
Kawasaki Disease is an acute, systemic vasculitis that primarily affects children, characterized by inflammation of the medium-sized arteries, particularly the coronary arteries. While the exact cause remains unknown, early diagnosis is critical to prevent long-term cardiovascular complications. At DiseaseMaps.org, 351 people with Kawasaki Disease have joined our community, sharing their experiences to help families navigate the complexities of this diagnosis and its potential sequelae.
How is Kawasaki Disease diagnosed?
There is no single diagnostic test for Kawasaki Disease. Instead, physicians rely on clinical criteria, including a high fever lasting at least five days and the presence of at least four of the following five clinical features:
- Bilateral conjunctival injection (red eyes without discharge).
- Changes in the oral mucosa, such as "strawberry tongue" or cracked lips.
- Polymorphous rash over the body.
- Changes in the extremities, such as redness or swelling of the hands and feet.
- Cervical lymphadenopathy (swollen lymph nodes, usually on one side).
What are the long-term risks of Kawasaki Disease?
The most significant concern for patients with Kawasaki Disease is the development of coronary artery aneurysms. With timely treatment using intravenous immunoglobulin (IVIG) and aspirin, the risk of developing these aneurysms is reduced from approximately 20-25% to less than 5%. Because Kawasaki Disease can impact the heart, regular echocardiograms are a standard part of the long-term management plan for those affected.
Next steps
- Consult a pediatric cardiologist immediately if you suspect symptoms of Kawasaki Disease.
- Keep a detailed log of the child's fever duration and physical symptoms to assist your medical team.
- Join the 351 members on DiseaseMaps.org to share your journey and learn from others living with the effects of Kawasaki Disease.
- Ensure your healthcare provider follows the American Heart Association guidelines for the long-term monitoring of Kawasaki Disease.
Medical disclaimer: This information is for educational purposes only and does not constitute professional medical advice, diagnosis, or treatment.
References
- American Heart Association (AHA) - Kawasaki Disease Clinical Guidelines
- NIH Genetic and Rare Diseases (GARD) Information Center - Kawasaki Disease
- Orphanet: Rare Disease Database - Kawasaki Disease (ORPHA:487)
- World Health Organization (WHO) - International Classification of Diseases (ICD)
