So what did she have?

by Bob Stuntz


Alright Dr. Shafer, what is the answer to your 2 year old with a fever?

As was well pointed out in one of the comments, the differential diagnosis includes scarlet fever, rubella, and Kawasaki’s disease, as well as toxic shock syndrome.  The patient clinically does not likely have rubella, as she is up to date on her immunizations, and kids should get their first MMR between 12 and 15 months of age, plus the clinical picture does not really fit.  Labs were drawn, and a throat culture was taken.  Rapid strep was negative, with a subsequent negative culture, and labs (CBC, CMP, LDH, and CRP) revealed the following abnormalities: LDH 360, CRP 8.4, AST 107, ALT 67.  Given the concern for possible Kawasaki’s disease and abnormal labs, she was admitted to pediatrics.  On day five, she continued to develop high fevers which became unresponsive to acetaminophen  and additionally developed edema of her hands and feet. On day six she was given IVIG and high-dose aspirin. Within hours after her infusion of IVIG, her fevers resolved and by day seven she began tolerating PO, rash had almost completely disappeared, and her hands and feet were less edematous than the day prior. By day 8 the child was fever free for 24 hours and she was discharged home. Her cardiac echo was negative for coronary artery aneurysm.  So, the answers are as follows:

A) What is the diagnosis: Kawasaki Disease

B) What additional tests outside of the above need to be done: Echocardiogram

C) What is the pathology, treatment, and disposition: Pathology includes a progression of arterial lesions with accompanying vasculitis, endothelial activation and injury. The cause of Kawasaki’s remains unknown. Disposition includes hospital admission with IVIG and ASA therapy (see below)

D) Are mom and her unborn baby going to be ok: There are no reported incidences of this disease affecting pregnancy

Kawasaki’s disease was first noted  by Tomisaku Kawasaki in 1961.  He published the first Japanese case report of the disease in 1967 and by 1974 he published the first English language case report of 50 Japanese patients presenting with a complex of symptoms including fever and rash that was linked to coronary artery vasculitis. It is possible that this was a new disease that emerged in first in Japan and then emanated to the western world through Hawaii versus an established disease that  was previously misdiagnosed; regardless, it is now a well-known entity that is not defined by nation boundaries. Kawaski’s disease is defined by the American Heart Association Guidelines as fever for at least five days and four or more of five clinical criteria:

1.  Conjunctival injection

2.  Cervical lymphadenopathy

3.  Oral mucosal changes

4.  Polymorphous rash

5.  Swelling or redness of extremities.

A diagnosis could also be made if only 3 criteria with fever are present in the setting of coronary artery disease in kids.  

In children who do not meet all of the classic criteria (most common in children under one years of age), measurement of high ESR and/or CRP levels as well as slight elevations in serum transaminase levels indicate diagnosis of atypical forms of the disease. This is important to note as children with atypical forms of the disease have notoriously higher rates of coronary artery aneurysms if untreated.  Of all children diagnosed with the disease, 15-25% of children will develop coronary artery aneurysm as a sequela of the vasculitis if left untreated. First line treatment, which should be administered within ten days of symptom onset, is a single infusion of IVIG (evidence rating of A) given in conjunction with high-dose aspirin, divided into four doses (evidence rating of C). Low dose aspirin should then be continued for 6-8 weeks following onset of symptoms if no cardiac abnormalities are present and indefinitely if coronary sequelae develop. Of note, despite treatment, 3-5% of children will still develop coronary aneurysms. 

In our patient, she presented with four days of fever and 4/5 clinical criteria.  By day 5, even had she not developed hand lesions, she would have had all the criteria for diagnosis.  And anytime someone has a rash and fever, you know what the family is going to ask: “Is this contagious?”  You can tell mom she is going to be OK, and so will her unborn baby.  

Sources:

1) Burns, J et. al. Kawasaki Disease: A Brief History. Pediatrics. 2000; 106 (2): e27

2) Freeman et.al. Kawasaki Disease: Summary of American Heart Association Guidelines. American Family Physician. 2006; 74(7): 1141-1148.