Delayed Diagnosis of Kawasaki Disease

Get CME5 Days of Fever Alarm


Vigilance can be the most difficult part of our job.  After being inundated with kids who have all started back to school and swapped their various viruses with one another and now have new fevers (after having just got rid of their previous virus), it is easy to become complacent. So sometimes it is best to have some preset “alarms” that go off when certain criteria are met.  For instance, set an alarm that gets your attention anytime someone presents with “Fever for 5 days.”

While “Fever for 5 days” may be due to numerous conditions and should make you ponder infectious as well as oncologic processes, the one that needs specific consideration is Kawasaki Disease.  We have discussed Kawasaki Disease in the past (see Morsel), but this time let us focus on the aspects that may lead to a Delayed Diagnosis of Kawasaki Disease.


Kawasaki Disease (KD) Basics

  • KD is an acute febrile vasculitis that occurs in children.
  • It is the LEADING CAUSE OF ACQUIRED HEART DISEASE among children in North America and Japan.
  • The cause is currently unknown.
  • 85% of cases occur in children < 5 years of age (which means there are cases in older children).
  • Recurrence rate is low (~4%).


It’s a Clinical Diagnosis

  • The diagnosis of KD is considered confirmed by the presence of Fever for 5 or more days and 4 of the 5 following criteria, provided that the illness cannot be explained by other known disease (see Morsel for common mimics).
  1. Bilateral Conjunctival Injection
  2. Mucous Membrane Changes of the Upper Respiratory Tract: injected pharynx, fissured lips, strawberry tongue.
  3. Changes of the Peripheral Extermities: peripheral edema, peripheral erythema, periungual desquamation.
  4. Polymorphous Rash: it really can be almost any type of rash.
  5. Cervical Adenopathy
  • Incomplete Kawasaki Disease
  1. The child has prolonged fever, but only 2 or 3 of the characteristic features (so not a complete complement of criteria).
  2. While the child has an incomplete set of criteria, he/she has as the same risk for developing coronary artery aneurysms.
  3. When you are entertaining this possibility, lab values (ESR/CRP) can be helpful in risk stratify the patient.
  4. It may also be a case when you actually need an ECHO done in the ED to help determine the child’s management (See Newburger article for evaluation algorithm).

Timing is Everything

  • 20% – 25% will go on to develop coronary artery aneurysms if untreated.
  • Intravenous Immunoglobin (IVIG) in addition to Aspirin lower the rate of developing coronary artery aneurysms to 3% – 5%.
  • The benefit of IVIG and Aspirin is seen if it is administered within the first 10 days of illness!
  • When therapy is initiated after Day 10, the incidence of coronary artery aneurysms increases ~3 times (so 9%-15%).
  • Vascular injury is evident as easly as 1 week after onset of fever.
  • Ideally, treatment should be started on or before Day 7 of illness.


Potential Causes of Delayed Diagnosis of Kawasaki Disease

  • The diagnostic characteristics are not specific and overlap with other entities.
  • The diagnostic characteristics may be transient and do not have to be all present concurrently!
  • Incomplete Kawasaki Disease – come on this isn’t even fair!
  • Lack of vigilance!!  We need to keep it on the DDx list!

Who is at greatest risk for having Delayed Diagnosis of Kawasaki Disease?

  • Children < 6 months of age
  • Children who present with < 4 criteria (Incomplete KD)
  • Those who lived a greater distance from the clinical center – that makes sense. May have poorer access to care.
  • Specific clinical centers also had higher rates of delayed diagnosis – perhaps pointing towards physicians not considering the Dx as much as other centers.

 Moral of the Story:

Stay vigilant.  When someone presents with a report of prolonged fever, reconsider Kawasaki Disease and be especially keen to revisit the potential of KD in those who are < 6 months and in those who Incomplete KD may be the diagnosis.



Minich LL, et al. Delayed Diagnosis of Kawasaki Disease: What are the Risk Factors? Pediatrics. 2007; 120: e1434 – e1440.

Newburger, JW. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease. Circulation. 2004;110:2747-2771.

Burns JC, et al. Kawasaki Disease:  A Brief History. Pediatrics. 2000; 106: e27.

Sean M. Fox
Sean M. Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renowned educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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  1. Hi please help, my daughter developed KD like symptoms 4 weeks ago. Because some of the clinical symptoms were mild, a diagnosis of KD was missed. However my daughter had, rash, red eyes, swollen lymph node on one side, abdominal pain and now has skin peeling.
    What should I do, I am worried it was KD? Is it too late to get treated? Worried about heart complications.

    • It would be best to discuss with her pediatrician as I cannot give personal medical guidance out over the internet.
      Express your concerns to her pediatrician.
      All the best,

  2. My daughter 18 months, had symptoms of KD back in feb, at the same time as having her MMR jabs (so put down her illness and high temp to that).
    however after temp, rash and general illness had gone, she was left with severe peeling of hands and feet.
    I took her to the doctors but they thought she seemed well at this point, but had a sore throat and peeling hands and feet, advised it could be streptococcal infection (no bloods taken) Advised to bring her back if not better (did mention symptoms of Kawasaki disease – but didn’t think it was), a week later she seemed well but wasn’t eating as normal so took her back to check her throat was no worse and doctor sent me to the hospital to rule out Kawasaki.
    The hospital did not do any blood tests, they took heart rate and temp and talked her through her history/illness – they concluded that they didn’t think it was Kawasaki and it might just be one of those things we never know what it was.
    Do you think I should be worried, if it was Kawasaki or covid 19, could she have underlying heart problems that we are in aware of? She seems completely well and back to her normal self now?

  3. What if a 18 month old had symptoms 3-4 weeks ago, should they get treatment now?

    • Good question.
      And speaking only hypothetically (as I cannot give advice about specific cases), I would think it unusual if there was complete resolution of symptoms (particularly fever) and no development of peeling skin for it to be KD. That being said, best to discuss with local ID team.
      Hope that that helps,

  4. I am an adult female a African American, I was diagnose with KD in 2011, this virus gave my body hell, I aged, severe stomach cramps diarrhea strawberry tongue you name my body went thru it. My doctor was at his sights end trying to figure out how I caught this especially since its known to be in young children of asian families.

    This week on CNN they were talking about Kawasaki and covid-19 being of the same virus family. I’m really scared especially since I know what I went thru.

    Anyone reading this that have medical information that you can share with me please do so, no jokes please I’m serious.

    • KD has never been clearly linked to one specific virus. It has, at times, been associated with various viral infections. Some of the inflammatory factors that we see with COVID, certainly can also be seen with KD though.
      More studies need to be done before a clear, definitive statement can be made.
      All the best,

  5. […] fever in the Pediatric Patient should cause you to think “outside of the box.”  Kawasaki Disease often comes to mind.  Naturally malignancy should also be considered and a thorough history (any […]

  6. […] Morsels is Vigilance: many potentially severe conditions present similarly to benign illnesses.  Kawasaki Disease, Rhabdomyolysis, Crohn’s Disease, and Compartment Syndrome are conditions that come to mind; […]

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