top of page

Guide to Kawasaki Disease for Pediatric Patients

  • Writer: Mayta
    Mayta
  • Jul 19, 2024
  • 5 min read



A Table for Diagnosis and Management of Kawasaki Disease

Diagnosis Criteria

Criteria

Description

Number of Criteria Required

Fever

Persistent fever lasting ≥ 5 days

1 (mandatory)

Erythematous Rash

Rash on the trunk and extremities, typically maculopapular and not vesicular


Bilateral Non-Exudative Conjunctivitis

Redness of both eyes without pus or discharge, with perilimbal sparing


Extremity Changes

Redness, swelling, and desquamation of hands and feet


Oral Changes

Red, cracked lips, strawberry tongue, and diffuse erythema of the oral and pharyngeal mucosa


Unilateral Cervical Lymphadenopathy

Enlargement of a single lymph node larger than 1.5 cm in diameter


Complete KD Diagnosis

Fever ≥ 5 days + 4 of the 5 clinical criteria listed above

4

Incomplete Kawasaki Disease Lab Criteria

Laboratory Test

Criteria

Number of Criteria Required

ESR or CRP

Elevated ESR (≥ 40 mm/hr) or CRP (≥ 3 mg/dL)

1 (mandatory)

Additional Lab Criteria

If ESR or CRP is elevated, 3 or more of the following:

3 out of 6

- Anemia for age



- White blood cell (WBC) count

≥ 15,000


- Platelet count

≥ 450,000 (typically after the first week)


- Elevated alanine transaminase (ALT)



- Serum albumin

< 3 g/dL


- Urine WBC

≥ 10 cells/high power field (HPF)


Management Dose

Treatment

Dosage

Notes

Intravenous Immunoglobulin (IVIg)

2 g/kg as a single infusion

Administer within the first 10 days of illness to reduce risk of coronary artery aneurysms.

High-Dose Aspirin

80-100 mg/kg/day divided into four doses

Until fever subsides for at least 48 hours.

Moderate-Dose Aspirin

30-50 mg/kg/day

If high-dose aspirin is not tolerated.

Low-Dose Aspirin

3-5 mg/kg/day

Continued for 6-8 weeks if no coronary abnormalities on follow-up echocardiograms.

Refractory KD Management

Treatment

Dosage

Notes

Second IVIg Dose

2 g/kg

For persistent fever ≥ 36 hours after the initial IVIg infusion.

Corticosteroids

Pulse methylprednisolone 30 mg/kg/day for 3 days

Used if refractory to IVIg.

Biologic Agents

Varies (e.g., infliximab, anakinra)

For cases refractory to IVIg and corticosteroids.

Summary Table for Management

Phase

Treatment

Dosage

Duration/Notes

Acute Phase

IVIg

2 g/kg

Single infusion within the first 10 days.


High-Dose Aspirin

80-100 mg/kg/day (divided into 4 doses)

Until fever subsides for at least 48 hours.


Moderate-Dose Aspirin

30-50 mg/kg/day

If high-dose aspirin is not tolerated.

Subacute Phase

Low-Dose Aspirin

3-5 mg/kg/day

Continued for 6-8 weeks if no coronary abnormalities.

Refractory KD

Second IVIg Dose

2 g/kg

If persistent fever ≥ 36 hours after initial IVIg.


Corticosteroids

Pulse methylprednisolone 30 mg/kg/day for 3 days

For refractory cases.


Biologic Agents

Varies

Used in refractory cases to IVIg and corticosteroids.



 

Definition

Kawasaki Disease (KD), also known as mucocutaneous lymph node syndrome, is an acute, self-limited vasculitis of medium-sized arteries. It predominantly affects children under the age of 5 and can lead to significant cardiovascular complications, notably coronary artery aneurysms if not promptly treated.

Epidemiology

Kawasaki Disease is most prevalent in children younger than 5 years, with the highest incidence in those under 2 years. In Thailand, the incidence is approximately 15 per 100,000 children. It occurs worldwide but is more common in Asian countries, particularly Japan.

Pathophysiology

The exact etiology of KD remains unknown, though it is believed to be triggered by an infectious agent in genetically predisposed individuals. The disease leads to widespread inflammation of medium-sized arteries, particularly the coronary arteries, causing aneurysms and other cardiovascular complications.


 

Clinical Presentation

Complete Kawasaki Disease

The diagnosis of complete KD is based on the presence of a fever lasting ≥ 5 days plus at least four of the following five clinical features:

  1. Erythematous Rash:

    • Typically, it appears on the trunk and extremities.

    • Characteristically maculopapular and not vesicular.

    • Rash may be polymorphous, including urticarial, erythema multiforme-like, or scarlatiniform.

  2. Bilateral Non-Exudative Conjunctivitis:

    • Redness of both eyes without pus or discharge.

    • Perilimbal sparing (the area around the iris is less inflamed).

  3. Extremity Changes:

    • Redness and swelling of the hands and feet.

    • Desquamation (peeling) of the fingers and toes usually occurs in the subacute phase.

  4. Oral Changes:

    • Red, cracked lips, strawberry tongue (red tongue with prominent papillae), and diffuse erythema of the oral and pharyngeal mucosa.

    • These changes are often among the earliest symptoms.

  5. Unilateral Cervical Lymphadenopathy:

    • Typically, it involves a single lymph node larger than 1.5 cm in diameter.

 

Incomplete Kawasaki Disease

Incomplete KD should be suspected in children with a prolonged fever (≥ 5 days) and 2-3 of the above clinical criteria. Laboratory findings support the diagnosis:

  • Elevated Erythrocyte Sedimentation Rate (ESR) ≥ 40 mm/hr or C-Reactive Protein (CRP) ≥ 3 mg/dL.

  • If either ESR or CRP is elevated, look for three or more of the following lab criteria:

    • Anemia for age.

    • White blood cell (WBC) count ≥ 15,000.

    • Platelet count ≥ 450,000 (typically after the first week).

    • Elevated alanine transaminase (ALT).

    • Serum albumin < 3 g/dL.

    • Urine WBC ≥ 10 cells/high power field (HPF).

In cases where laboratory criteria are not conclusive, an echocardiogram may help diagnose KD by revealing coronary artery abnormalities.

 

Natural Course

Kawasaki Disease typically progresses through three distinct phases:

  1. Acute Phase (1-2 weeks):

    • High fever persisting for ≥ 5 days.

    • Rash, conjunctivitis, oral changes, cervical lymphadenopathy, and extremity swelling.

    • Laboratory findings may show elevated inflammatory markers (ESR, CRP), sterile pyuria, elevated liver enzymes, and hypoalbuminemia.

  2. Subacute Phase (2-4 weeks):

    • Resolution of fever and acute symptoms.

    • Desquamation of fingers and toes.

    • Thrombocytosis and increased risk of coronary artery aneurysms.

  3. Convalescent Phase (4-8 weeks):

    • Symptoms resolve, and lab values normalize.

    • Coronary aneurysms may still develop or resolve during this phase.

    • Follow-up echocardiography is crucial to monitor coronary artery status.

 

Diagnosis

Diagnosing KD requires a high index of suspicion, especially in children with prolonged fever and incomplete criteria. The diagnostic approach involves:

  1. Clinical Criteria: As detailed above, considering complete and incomplete KD criteria.

  2. Laboratory Tests:

    • Complete Blood Count (CBC): Elevated WBC, anemia, thrombocytosis.

    • Inflammatory markers: Elevated ESR and CRP.

    • Liver function tests: Elevated ALT.

    • Urinalysis: Sterile pyuria.

  3. Echocardiography:

    • Essential for all suspected cases to assess coronary artery involvement.

    • Repeat echocardiograms are typically performed at diagnosis, 2 weeks, and 6-8 weeks after onset.

 

Differential Diagnosis

Kawasaki Disease must be differentiated from other febrile illnesses with similar presentations, including:

  • Multisystem Inflammatory Syndrome in Children (MIS-C):

    • Associated with COVID-19.

    • Presents with fever, gastrointestinal symptoms, rash, conjunctivitis, and cardiovascular involvement.

    • Key differentiators: History of COVID-19 exposure, more prominent gastrointestinal symptoms, and often more severe myocardial involvement.

  • Scarlet Fever:

    • Caused by Group A Streptococcus.

    • Features include a sandpaper-like rash, fever, strawberry tongue, and Pastia lines.

    • The presence of exudative pharyngitis and positive rapid streptococcal test help differentiate.

  • Measles:

    • Caused by the measles virus.

    • Characterized by a maculopapular rash, Koplik spots, conjunctivitis, and cough.

    • History of exposure and lack of vaccination are key clues.

  • Toxic Shock Syndrome:

    • Caused by Staphylococcus aureus or Streptococcus pyogenes toxins.

    • Features include high fever, diffuse rash, desquamation, hypotension, and multi-organ involvement.

    • Differentiated by rapid progression and presence of shock.

  • Stevens-Johnson Syndrome:

    • Severe mucocutaneous reaction often triggered by medications or infections.

    • Characterized by extensive mucosal involvement and skin detachment.

    • Differentiated by more severe mucocutaneous involvement and targetoid lesions.

  • Viral Exanthems:

    • Various viruses can cause fever and rash, such as enteroviruses and adenoviruses.

    • Differentiated by milder clinical course and less consistent laboratory findings.

 

Management

Specific Treatment

  • Intravenous Immunoglobulin (IVIg):

    • Dosage: 2 g/kg as a single infusion, administered within the first 10 days of illness.

    • Benefit: Reduces the risk of coronary artery aneurysms from 20-25% to <5%.

    • Administration: Start with a low infusion rate and increase gradually to minimize the risk of adverse reactions. Monitor for signs of anaphylaxis, such as fever, chills, or hypotension.

  • Aspirin Therapy:

    • High-Dose Aspirin: 80-100 mg/kg/day divided into four doses until the fever subsides for at least 48 hours.

    • Moderate-Dose Aspirin: 30-50 mg/kg/day may be used if high-dose is not tolerated.

    • Low-Dose Aspirin: 3-5 mg/kg/day continued for 6-8 weeks if there are no coronary abnormalities on follow-up echocardiograms.

 

Refractory KD

  • IVIg-Resistant KD: Persistent fever ≥ 36 hours after IVIg infusion. Options include:

  • Second IVIg Dose: 2 g/kg.

  • Corticosteroids: Pulse methylprednisolone 30 mg/kg/day for 3 days or longer courses based on clinical judgment.

  • Biologic Agents: Such as infliximab or anakinra, in cases refractory to IVIg and corticosteroids.

 

Follow-Up and Monitoring

Regular follow-up is critical to monitor for cardiac complications:

  • Echocardiography: Initial, 2 weeks, and 6-8 weeks post-diagnosis.

  • Cardiology Consultation: For cases with coronary abnormalities.

  • Long-Term Follow-Up: For children with coronary artery changes, regular follow-up with a cardiologist is essential.

Vaccination Considerations

  • Live Vaccines: Postpone for 11 months after IVIg due to potential interference with vaccine efficacy.

  • Affected Vaccines: JE (Japanese Encephalitis), VZV (Varicella Zoster Virus), MMR (Measles, Mumps, Rubella).

  • Revaccination: If administered within 14 days before IVIg, revaccinate 11 months later.

 

Summary

Kawasaki Disease is a serious pediatric condition requiring prompt recognition and intervention to prevent coronary artery complications. The cornerstone of treatment is IVIg and aspirin, with adjustments for refractory cases. Echocardiography and regular follow-up are essential to ensure the resolution of symptoms and monitor for long-term cardiovascular outcomes. Pediatric residents should maintain a high index of suspicion for KD in children with prolonged fever and use a systematic approach to diagnose and manage this condition effectively.

Recent Posts

See All
OSCE: Cervical Punch Biopsy

Introduction A cervical punch biopsy is a procedure used to obtain a small tissue sample from the cervix to investigate suspicious...

 
 
 

Komentáře

Hodnoceno 0 z 5 hvězdiček.
Zatím žádné hodnocení

Přidejte hodnocení
Post: Blog2_Post

Message for International Readers
Understanding My Medical Context in Thailand

By Uniqcret, M.D.
 

Dear readers,
 

My name is Uniqcret, which is my pen name used in all my medical writings. I am a Doctor of Medicine trained and currently practicing in Thailand, a developing country in Southeast Asia.
 

The medical training environment in Thailand is vastly different from that of Western countries. Our education system heavily emphasizes rote memorization—those who excel are often seen as "walking encyclopedias." Unfortunately, those who question, critically analyze, or solve problems efficiently may sometimes be overlooked, despite having exceptional clinical thinking skills.
 

One key difference is in patient access. In Thailand, patients can walk directly into tertiary care centers without going through a referral system or primary care gatekeeping. This creates an intense clinical workload for doctors and trainees alike. From the age of 20, I was already seeing real patients, performing procedures, and assisting in operations—not in simulations, but in live clinical situations. Long work hours, sometimes exceeding 48 hours without sleep, are considered normal for young doctors here.
 

Many of the insights I share are based on first-hand experiences, feedback from attending physicians, and real clinical practice. In our culture, teaching often involves intense feedback—what we call "โดนซอย" (being sliced). While this may seem harsh, it pushes us to grow stronger, think faster, and become more capable under pressure. You could say our motto is “no pain, no gain.”
 

Please be aware that while my articles may contain clinically accurate insights, they are not always suitable as direct references for academic papers, as some content is generated through AI support based on my knowledge and clinical exposure. If you wish to use the content for academic or clinical reference, I strongly recommend cross-verifying it with high-quality sources or databases. You may even copy sections of my articles into AI tools or search engines to find original sources for further reading.
 

I believe that my knowledge—built from real clinical experience in a high-intensity, under-resourced healthcare system—can offer valuable perspectives that are hard to find in textbooks. Whether you're a student, clinician, or educator, I hope my content adds insight and value to your journey.
 

With respect and solidarity,

Uniqcret, M.D.

Physician | Educator | Writer
Thailand

bottom of page