SARS-CoV-2-induced Kawasaki-like hyperinflammatory syndrome seems to be caused by a delayed response to SARS-CoV-2. It resembles Kawasaki disease complicated by macrophage activation syndrome, although it has peculiar features, such as prodromal diarrhea, capillary leak syndrome, and myocardial dysfunction.
The most significant clinical difference between the 2 conditions is the type of cardiac involvement seen. Whereas the most well-recognized sequelae of KD is coronary artery changes, in MIS-C there is prominent myocardial involvement, with decreased ventricular function seen commonly.
The clinical features of Kawasaki disease can be mistaken for other illnesses such as streptococcal or staphylococcal infections (scarlet fever or toxic shock syndrome), parasite or viral infections (leptospirosis, measles, or adenovirus), and drug reactions (Stevens-Johnson syndrome).
There are two forms of KD: complete and incomplete. Diagnosis of complete KD requires fever of at least 5 days' duration along with 4 or 5 of the principal clinical features.
What causes the inflammation in Kawasaki disease remains unknown. It is most likely an abnormal response by the immune system of some children to a common germ. Kawasaki disease is not contagious to other children.
The most feared complication of KD is coronary artery abnormality development, and patients with atypical KD are also at risk. Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children.
Kawasaki disease commonly leads to inflammation of the coronary arteries, which supply oxygen-rich blood to the heart. Kawasaki disease was previously called mucocutaneous lymph node syndrome because it also causes swelling in glands (lymph nodes) and mucous membranes inside the mouth, nose, eyes and throat.
Kawasaki disease is not well understood and the cause is yet unknown. It may be an autoimmune disorder. The problem affects the mucous membranes, lymph nodes, walls of the blood vessels, and the heart.
Long-term effects of Kawasaki disease, however, can include heart valve issues, abnormal heartbeat rhythm, inflammation of the heart muscle, and aneurysms (bulges in blood vessels). These lasting heart conditions are rare. Less than 2% of patients experience coronary artery enlargement that carries over into adulthood.
Kawasaki disease is characterized by acute vasculitis and inflammation that can affect the coronary arteries. Anti-inflammatory medicine is effective in the acute stages of the disease. What is new: Children with Kawasaki disease have an increased risk of developing autoimmune disease in the long term.
Kawasaki disease is frequently misdiagnosed or not diagnosed right away. Since early treatment and intervention is key to avoiding long-term heart damage from Kawasaki disease, these types of diagnostic errors can have serious consequences.
Aneurysm. Inflammation in the blood vessels that supply blood to the heart (coronary arteries) can cause a section of the artery wall to weaken. As the blood passes through the weakened part of the artery wall, the blood pressure causes it to bulge outwards like a balloon. This is called an aneurysm.
In the United States, children with Kawasaki disease are treated initially with a single dose of IVIG (2 g per kg) and high-dose aspirin (80 to 100 mg per kg per day, divided into four doses).
Several cases of a severe multi-system inflammatory syndrome have been reported in children during the current COVID-19 pandemic. Clinical manifestations may resemble Kawasaki disease (KD) which is the most common childhood vasculitis. A 7-year-old-girl presented with fever, rash and abdominal pain.
MIS-C causes different internal and external body parts to become inflamed, including the heart, lungs, kidneys, brain, skin, eyes, or gastrointestinal tract. MIS-C can be serious, even deadly, but most children who are diagnosed with this condition get better with medical care.
Kawasaki disease can also affect other parts of the body, including the brain and nervous system, the immune system, and the digestive system.
Kawasaki disease often goes away on its own, but if it is not treated it can cause serious injury to the heart and other organs. In some cases, the disease can affect the coronary arteries, which are blood vessels that supply oxygen-rich blood to the heart. This can cause serious heart problems.
Kawasaki disease can't be prevented. Children can make a full recovery within 6 to 8 weeks if it's diagnosed and treated promptly, but complications can develop. It's important to see a GP and start treatment as soon as possible. Read more about diagnosing Kawasaki disease.
In conclusion, the multisystemic inflammatory syndrome in children (MIS-C) related to the SARS-CoV-2 pandemic (also termed Kawasaki-like disease, or Kawa-COVID-19) appears to share clinical, pathogenetic and laboratory features with KD, toxic shock syndrome, and MAS.
Kawasaki disease (KD), particularly its severe form – Kawasaki disease shock syndrome (KDSS) – is rare in adults. Some recent reports have shown that COVID-19 is associated with the appearance of cases of Kawasaki-like disease (KLD), particularly in children.
Experts do not know exactly what causes Kawasaki disease. It is not contagious, so it cannot spread from person to person. It might be the result of changes to certain genes or related to viral or bacterial infections.
“It's concerning that high consumption of foods like tofu, soy formula, edamame and other soy products is a risk factor for Kawasaki disease,” said Dr. Michael Portman, a pediatric cardiologist and researcher at Seattle Children's Research Institute who was the lead author of the study.
The course of Kawasaki disease can be divided into three clinical phases: acute, subacute and convalescent.
Survival ranged from 92% to 99% at 10 years, 85% to 99% at 20 years, and 88% to 94% at 30 years. MACE-free survival, mostly studied in those with CAA, varied from 66% to 91% at 10 years, 29% to 74% at 20 years, and 36% to 96% at 30 years.