It is a type of vasculitis. Ethnic background: People of Asian ancestry, specifically Japanese or Chinese, and Black Americans are more susceptible to Kawasaki disease; Genetics: If the parents had Kawasaki disease… Kawasaki disease is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age. [97][98][99][100] A plausible explanation is that it may be caused by an infection that triggers an inappropriate immunologic cascade in a small number of genetically predisposed children. adenovirus, enterovirus); staphylococcal and streptococcal toxin-mediated diseases such as scarlet fever and toxic shock syndrome; drug hypersensitivity reactions (including Stevens Johnson syndrome); systemic onset juvenile idiopathic arthritis; Rocky Mountain spotted fever or other rickettsial infections; and leptospirosis. The main symptoms were shock, unrest, vomiting, and abdominal pain; chest pain was most common in older children. [1] If the coronary arteries are involved, ongoing treatment or surgery may occasionally be required. described the same illness in 16 children in Hawaii. [12], Kawasaki disease is rare. Mayo Clinic does not endorse companies or products. [7], Based on clinical findings, a diagnostic distinction may be made between the 'classic' / 'typical' presentation of Kawasaki disease and 'incomplete' / 'atypical' presentation of a "suspected" form of the disease. "Mayo," "Mayo Clinic," "MayoClinic.org," "Mayo Clinic Healthy Living," and the triple-shield Mayo Clinic logo are trademarks of Mayo Foundation for Medical Education and Research. [29], Cervical lymphadenopathy is seen in 50% to 75% of children, whereas the other features are estimated to occur in 90%,[14][22] but sometimes it can be the dominant presenting symptom. [8] Timely diagnosis requires careful history-taking and thorough physical examination. Kawasaki disease. [115] Gene–gene interactions also seem to affect susceptibility and prognosis. Kawasaki Disease Symptoms. One or more changes of the arms and legs, including redness, Disease cannot be explained by some other known disease process, †A diagnosis of Kawasaki disease can be made if fever and only three changes are present if coronary artery disease is documented by two-dimensional. [39][40] It can be polymorphic, not itchy, and normally observed up to the fifth day of fever. [83] It can also be found as necrotizing vasculitis, progressing into peripheral gangrene. A number of theories link the disease to bacteria, viruses or other environmental factors, but none has been proved. [127][128], Other diseases involving necrotizing vasculitis include polyarteritis nodosa, granulomatosis with polyangiitis, Henoch–Schönlein purpura, and eosinophilic granulomatosis with polyangiitis. Ethnicity. Kliegman RM, et al. [5][13], Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen. Elsevier; 2020. https://www.clinicalkey.com. Kawasaki disease affects boys more than girls, with people of Asian ethnicity, particularly Japanese people. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of nontreatment. ", "Understanding SARS-CoV-2-related multisystem inflammatory syndrome in children", "A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis", "Association of Kawasaki disease with tropospheric wind patterns", "Kawasaki disease and ENSO-driven wind circulation", "Infectious disease: Blowing in the wind", "Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan", "Dissecting Kawasaki disease: a state-of-the-art review", "Kawasaki Disease: Global Burden and Genetic Background", "Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease", "EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides", "Kawasaki disease – Diagnosis and treatment", "Kawasaki disease and immunisation: Standardised case definition & guidelines for data collection, analysis", "Multisystem inflammatory syndrome in children and adolescents with COVID-19", "Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with Coronavirus Disease 2019 (COVID-19)", "SARS-CoV-2-Related Inflammatory Multisystem Syndrome in Children: Different or Shared Etiology and Pathophysiology as Kawasaki Disease? [127] Systemic vasculitides may be classified according to the type of cells involved in the proliferation, as well as the specific type of tissue damage occurring within the vein or arterial walls. Incidence rates vary between countries. [6] It is a form of vasculitis, where blood vessels become inflamed throughout the body. Vasculitis. [30] Affected lymph nodes are painless or minimally painful, nonfluctuant, and nonsuppurative; erythema of the neighboring skin may occur. [4][124][125], Debate has occurred about whether Kawasaki disease should be viewed as a characteristic immune response to some infectious pathogen, as an autoimmune process, or as an autoinflammatory disease (i.e. Check out these best-sellers and special offers on books and newsletters from Mayo Clinic. [161], In Japan, the rate is 240 in every 100,000 people. [14], Even when treated with high-dose IVIG regimens within the first 10 days of illness, 5% of children with Kawasaki disease develop at the least transient coronary artery dilation and 1% develop giant aneurysms. Kawasaki disease affects boys more than girls, with people of Asian ethnicity, particularly Japanese and Korean people, most susceptible, as well as people of Afro-Caribbean ethnicity. The good news is that Kawasaki disease is usually treatable, and most children recover from Kawasaki disease without serious problems. [138] Aspirin therapy is started at high doses until the fever subsides, and then is continued at a low dose when the patient returns home, usually for two months to prevent blood clots from forming. Mayo Clinic; 2018. [123], The broadness of the differential diagnosis is a challenge to timely diagnosis of Kawasaki disease. In the United States, the disease has been reported in all racial and ethnic groups but occurs most often among children of Asian-American descent. [30] In developed nations, it appears to have replaced acute rheumatic fever as the most common cause of acquired heart disease in children. While the use of TNF alpha blockers (TNF-α) may reduce treatment resistance and the infusion reaction after treatment initiation, further research is needed. [citation needed], Laboratory evidence of increased inflammation combined with demographic features (male sex, age less than six months or greater than eight years) and incomplete response to IVIG therapy create a profile of a high-risk patient with Kawasaki disease. Kawasaki disease is a leading cause of acquired heart disease in children. [21], Bilateral conjunctival inflammation has been reported to be the most common symptom after fever. [27] Keratic precipitates are another eye manifestation (detectable by a slit lamp, but are usually too small to be seen by the unaided eye). [29] The mucosa of the mouth and throat may be bright red, and the tongue may have a typical "strawberry tongue" appearance (marked redness with prominent gustative papillae). Kawasaki disease is a pediatric illness and over 80% of cases occur in patients under the age of 5 years, with nearly all cases occurring by the age of 8 years. [citation needed], Rarely, recurrence can occur in Kawasaki disease with or without treatment.[153][154]. Accessed Sept. 3, 2019. [112] The associated genes and their levels of expression appear to vary among different ethnic groups, both with Asian and non-Asian backgrounds. Inflammation of the coronary arteries can lead to weakening and bulging of the artery wall (aneurysm). Aug. 5, 2A019. Objective Kawasaki disease (KD) is an increasingly common vasculitis with risk of coronary artery aneurysms (CAAs). [113] Genome-wide association studies and studies of individual candidate genes have together helped identify specific single nucleotide polymorphisms (SNPs), mostly found in genes with immune regulatory functions. However, a Cochrane review published in 2017 found that, in children, the use of corticosteroids in the acute phase of KD was associated with improved coronary artery abnormalities, shorter hospital stays, a decreased duration of clinical symptoms, and reduced inflammatory marker levels. [59] The highest rate of progression to stenosis occurs among those who develop large aneurysms. Because children with Kawasaki disease will be taking aspirin for up to several months, vaccination against varicella and influenza is required, as these infections are most likely to cause Reye syndrome. [136], Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some)[137] but salicylates alone are not as effective as IVIG. Mayo Clinic. This content does not have an Arabic version. [60] The highest risk of MI occurs in the first year after the onset of the disease. [15][16] Recently, it is reported to be present in patients with atypical or incomplete Kawasaki disease;[17][18] nevertheless, it is not present in 100% of cases. [169] In particular, old pathological descriptions from Western countries of infantile polyarteritis nodosa coincide with reports of fatal cases of Kawasaki disease. Mayo Clinic is a not-for-profit organization. [170], Kawasaki-like disease temporally associated with COVID-19. Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, acc… [63] Some of these lesions require valve replacement. IVIG given within the first 10 days of the disease reduces the risk of damage to the coronary arteries in children, without serious adverse effects. [24] This usually begins shortly after the onset of fever during the acute stage of the disease. Ferri FF. https://www.uptodate.com/contents/search. Kawasaki disease (KD) is a severe acute inflammation of blood vessels throughout the body characterized by a prolonged fever. Three things are known to increase your child's risk of developing Kawasaki disease. A physical examination will demonstrate many of the features listed above. Newsletter: Mayo Clinic Health Letter — Digital Edition, FREE book offer – Mayo Clinic Health Letter. There is limited evidence to indicate whether children should continue to receive salicylate as part of their treatment. Treatment with IVIG can cause allergic and nonallergic acute reactions, aseptic meningitis, fluid overload, and rarely, other serious reactions. Vasculitis means inflammation of the blood vessels. Kawasaki disease is an acute inflammatory vasculitis of medium size arteries that occurs mainly in children aged 6 months to 5 years but can occur at any age, including younger infants, and even occasionally in adults.1 2 3 Although one or multiple infectious triggers are most likely, the precise cause is unclear. No one knows what causes Kawasaki disease, but scientists don't believe the disease is contagious from person to person. Learn about a condition that causes similar symptoms to Kawasaki disease called MIS-C, Vaccine updates, safe care and visitor guidelines, and trusted coronavirus information, Mayo Clinic Graduate School of Biomedical Sciences, Mayo Clinic School of Continuous Professional Development, Mayo Clinic School of Graduate Medical Education, See our safety precautions in response to COVID-19, Book: Mayo Clinic Family Health Book, 5th Edition. It can also affect the blood vessels supplying the heart muscle (coronary arteries). [7], In 2020, reports of a Kawasaki-like disease following exposure to SARS-CoV-2, the virus responsible for COVID-19, emerged in the US and Europe. McCrindle BW, et al. In: Cassidy JT, Petty RE, eds. Cassidy JT, Petty RE. [102] There has been debate as to whether the infectious agent might be a superantigen (i.e. It is more common in boys, and in children of North Asian ethnicity, but is seen in all ethnic groups. [127] Under this classification scheme for systemic vasculitis, Kawasaki disease is considered to be a necrotizing vasculitis (also called necrotizing angiitis), which may be identified histologically by the occurrence of necrosis (tissue death), fibrosis, and proliferation of cells associated with inflammation in the inner layer of the vascular wall. Sundel R. Kawasaki disease: Initial treatment and prognosis. [25][26] Iritis can occur, too. [19], The first day of fever is considered the first day of the illness,[14] and its duration is typically one to two weeks; in the absence of treatment, it may extend for three to four weeks. Kawasaki Disease Foundation. [110] One source has been suggested in northeastern China. [38] The rash varies over time and is characteristically located on the trunk; it may further spread to involve the face, extremities, and perineum. At risk of coronary artery aneurysms after Kawasaki disease and how it can be made purely the... Susceptibility to Kawasaki disease incidence occurs in the World symptoms show up in phases endothelial.... Us by up to the Terms and conditions and Privacy Policy linked below bulging of the abrupt onset the! Tests may support the diagnosis kawasaki disease ethnicity ubiquitous virus, [ 106 ] possibly one enters... Diagnosis requires careful history-taking and thorough physical examination will demonstrate many of the most commonly diagnosed vasculitis... 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