Hello everyone, today I will be discussing Kawasaki Disease, its signs and symptoms, Diagnosis, and Treatment. Kawasaki is an acute idiopathic medium-sized muscular vasculitis. It is a rare heart condition whose cause is unknown that causes the inflammation of medium-sized blood vessels. It was previously known as mucocutaneous lymph node syndrome, it is associated with fever and it affects children under the age of 5 years but can affect any age. It was first reported in 1961 by Dr. Tomisaku Kawasaki in Japan. The first case of Kawasaki disease was a four-year-old male patient which was presented with a high fever for about 2 weeks. The child demonstrated symptoms such as bilateral conjunctival hyperemia, bleeding lips, mucus membrane, strawberry-looking tongue, diffuse erythema of the oral cavity, and dried and reddish lips. In 5 years, Dr. Kawasaki observed 50 cases that had similar presentations. This led to the publishing of his paper titled "Acute febrile musculocutaneous lymph node syndrome: clinical observation of 50 cases" in 1967.
Kawasaki Disease can show complications when not treated. These complications include coronary artery aneurysms, Arrhythmias, peripheral artery occlusion, and heart failure. These complications make it very important to identify this disease at an early stage before it becomes a complicated issue. Kawasaki disease has a higher prevalence in Japan and other Asian countries with an incidence of about 239.6 per 100 000 children in the year 2010
The pathophysiology of Kawasaki disease involves CD8+ T cells, IgA+ plasma cells, eosinophils, and macrophages. Research shows that infections from staphylococcus, streptococcus, and virus can trigger the cause of this disease. The microorganism is then identified by antibodies which then give an inflammatory response which then attacks the small and medium vessels in the body including the coronary arteries. This inflammatory response damages the elastic fibers in the vessel which could lead to the complete loss of their normal structural integrity.
The clinical features of Kawasaki disease are fever and mucocutaneous inflammation. The fever is minimally responsive to anti-fever medications and remains high. The fever stays for above 5 days. Patients would have bilateral, non-exudative inflammation of the conjunctiva (conjunctivitis), which is common with patients with Kawasaki disease. The patient would have a polymorphous rash which begins a few days after the fever, Patients would have lymphadenopathy at the anterior cervical region. Patients also have a strawberry-like tongue (Mucositis), with cracked lips, patients exhibit changes in their extremities such as peeling of the skin from under their nails (Periumgual desquamation), Plantar erythema, edema of the feet and the hands, patients could experience Arthritis. Patients with Kawasaki disease would experience abdominal pain, nausea, rhinorrhea, Diarrhea, and cough. These clinical features may not manifest at once, but important things to take note of are fever which is above 5 days, conjunctivitis, rash, edema and extremity changes, Adenopathy, and Mucositis.
The risk factors of Kawasaki disease are;
To diagnose Kawasaki, there needs to be a fever lasting for at least five days with no other explanation for its consistent burning after using anti-fever medications. The patient has to manifest 4 out of all the symptoms or clinical features of Kawasaki disease. in cases of incomplete Kawasaki disease where patients would have had a fever for about 5 days and manifest 2 to 3 out of all the clinical manifestations, and an elevated C-Reactive Protein (CRP) or Erythrocyte Sedimentation (ESR). Laboratory findings can also help identify Kawasaki disease. Individuals with Kawasaki disease will have normocytic anemia, patients will have normal platelet at the onset but as the condition worsens, the level begins to increase (thrombocytosis), and patients will have increased leukocytosis, along with elevated C-Reactive Protein (CRP) or Erythrocyte Sedimentation (ESR), and elevated transaminase.
Treatment of Kawasaki disease includes the use of intravenous immunoglobin (IVIG) and aspirin. It is very important to check that there is no resistance to IVIG. When children have enlarged coronary arteries prior to the introduction of intravenous immunoglobin (IVIG), then there is a possibility of IVIG resistance. When children are less than six months of age, there are chances of IVIG resistance. When patients have macrophage activation syndrome, there will high chance of IVIG resistance.
Kawasaki is a systemic medium-sized vasculitis disease that affects children but can sometimes affect teenagers and adults. Kawasaki and Henoch-schonlein purpura are the most common medium-sized vasculitis disease in children and are the leading cause of childhood-acquired heart disease in developed countries. The symptoms of Kawasaki disease can be remembered with the mnemonic CREAM (Conjunctivitis without exudate, Rash, Edema or erythema of hands or feet, Adenopathy, and Mucosal erythema).
https://onlinelibrary.wiley.com/doi/10.1002/cti2.1284
https://www.nature.com/articles/s41584-020-0426-0
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3095860/
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/symptoms-causes/syc-20354598
https://www.nhs.uk/conditions/kawasaki-disease/
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374576/
