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Kawasaki Disease (“KD”) is a vasculitis (inflammation of blood vessels) of unknown cause. It is a rare disorder that affects mostly children, rarely adults, and has been linked to COVID-19 (coronavirus).
Typical symptoms of KD in both adults and children include fever, conjunctivitis (“red eyes”), pharyngitis (“red throat”), “strawberry” tongue, and an erythematous skin rash (“red skin”) on the palms and soles of the hands and feet and on the trunk and genitalia. Symptoms more common to adults include cervical adenopathy (93% of adults vs. 15% of children), hepatitis (65% vs. 10%), and arthritis (61% vs. 24–38%). Symptoms more likely to occur in children include meningitis (10% vs. 34%), thrombocytosis (high platelet count) (55% vs. 100%), and coronary artery aneurysms (5% vs. 18–25%). [Ref: Wolff A, et al. “Acute Kawasaki Disease: Not Just for Kids.” J Gen Intern Med. 2007 May; 22(5): 681–684.]
The vasculitis that occurs with KD causes inflammation and swelling of the affected blood vessels, which can lead to vascular obstruction, thrombosis (blood clots), aneurysm, or fibrosis (scarring).  Coronary artery aneurysms occur in approximately 18–25% of children with Kawasaki Disease, but only in about 5% of adult cases. Coronary aneurysms are serious and can cause heart attacks (myocardial infarction).  Id.
Aspirin, when started early, is quite effective in the treatment of Kawasaki Disease. It helps not only the symptoms of KD, but it also prevents the development of coronary artery aneurysms. Treatment with intravenous gamma globulin (IVIG) infusions is effective and is usually administered along with aspirin.
Corticosteroids have been shown to be effective when used, but typically they are not used to treat KD by most doctors. Aspirin therapy and IVIG are preferred.
COVID-19 and Kawasaki Disease
For reasons that are not completely understood, the coronavirus appears to be less likely to infect children. However, children and young adults are not immune from COVID-19, and there have been some very sick young children with COVID-19, some of whom have died.
The first published case of COVID-19 and KD was reported in a 6-month-old infant with classic Kawasaki disease (KD), who also screened positive for COVID-19, from Stanford Medical Center. [Ref:  Jones VG, et al. “COVID-19 and Kawasaki Disease: Novel Virus and Novel Case.” Hospital Pediatrics, 2020.]
Cases with KD/COVID-19 have been reported in New York, Delaware, Italy, Spain, and the UK. Children are typically 2 to 15 years old with fever, rash, abdominal pain, vomiting, diarrhea, cardiac inflammation and shock. Some have died. However, less than half have had respiratory symptoms.
It is unclear what the relationship is between COVID-19 and KD in these children. Doctors have been calling it “Pediatric Multi-System Inflammatory Syndrome Temporally Associated with COVID-19," until more is known.
Long-Term Disability in Kawasaki Disease
Diagnosis and treatment of KD in children and adults usually results in resolution of most of the symptoms within a few weeks to months. However, both in children and adults, there may be serious long-term cardiac involvement that can cause heart disease, myocardial infarction, and death.
While long-term disability (LTD) from KD is uncommon, it can occur – most often in persons who develop chronic heart disease.
At LawMed, we are continuing to monitor developments as the occur.
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