Kawasaki disease 1038491 224673236 2008-07-09T22:25:09Z 146.79.254.10 External linkt to heartcenter.seattlechildrens.org is now heart.seattlechildrens.org {{Refimprove|date=September 2007}} {{DiseaseDisorder infobox | Name = Kawasaki disease | ICD10 = {{ICD10|M|30|3|m|30}} | ICD9 = {{ICD9|446.1}} | ICDO = | Image = Verkalkte aneurysmatische Coronarien.jpg | Caption = Kawasaki syndrome | OMIM = 611775 | OMIM_mult = | MedlinePlus = 000989 | eMedicineSubj = med | eMedicineTopic = 1223 | eMedicineSubj = ped | eMedicineTopic = 1236 | DiseasesDB = 7121 | MeshID = D009080 | }} '''Kawasaki disease''', also known as '''lymph node syndrome''', '''mucocutaneous node disease''', '''infantile polyarteritis''' and '''Kawasaki syndrome''', is a poorly understood self-limited [[vasculitis]] that affects many organs, including the [[skin]] and [[mucous membrane]]s, [[lymph node]]s, [[blood vessel]] walls, and the [[heart]]. It does not seem to be contagious. It was first described in [[1967]] by Dr. Tomisaku Kawasaki in Japan.<ref>{{cite journal | author = Kawasaki T | title = [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children] | format=in Japanese | journal = Arerugi | volume = 16 | issue = 3 | pages = 178–222 | year = 1967 | pmid = 6062087}}</ref> ==Incidence and risk factors== By far, the highest incidence of Kawasaki disease occurs in [[Japan]] (175 per 100,000), though its incidence in the [[United States]] is increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex. Approximately 2000 cases are identified in the [[United States]] each year.<ref name="urlKawasaki Disease - Signs and Symptoms">{{cite web |url=http://www.ucsfhealth.org/childrens/medical_services/heart_center/acquired/conditions/kawasaki/signs.html |title=Kawasaki Disease - Signs and Symptoms |format= |work= |accessdate=}}</ref> ==Causes== The causative agent of Kawasaki disease is still unknown.<ref name="pmid18364728">{{cite journal |author=Rowley AH, Baker SC, Orenstein JM, Shulman ST |title=Searching for the cause of Kawasaki disease--cytoplasmic inclusion bodies provide new insight |journal=[[Nat. Rev. Microbiol.]] |volume=6 |issue=5 |pages=394–401 |year=2008 |month=May |pmid=18364728 |doi=10.1038/nrmicro1853}}</ref> However, current [[etiology|etiological]] [[theory|theories]] center primarily on [[immune system|immunological]] causes for the disease. Much research is being performed to discover a definitive [[toxin]] or [[antigen]]ic substance, possibly a [[superantigen]], that is the specific cause of the disease. An unknown virus may play a role as an inciting factor as well. An association with [[ITPKC]] has been identified.<ref name="pmid18084290">{{cite journal |author=Onouchi Y, Gunji T, Burns JC, ''et al'' |title=ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms |journal=Nat. Genet. |volume=40 |issue=1 |pages=35–42 |year=2008 |month=January |pmid=18084290 |doi=10.1038/ng.2007.59}}</ref> ==Presentation== The [[cardiac]] complications are, by far, the most important aspect of the disease. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the [[coronary arteries]] and subsequent [[coronary artery aneurysm]]s. These aneurysms can lead to [[myocardial infarction]] ([[myocardial infarction|heart attack]]) even in young children. Overall, about 10&ndash;18% of children with Kawasaki disease develop coronary artery aneurysms Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994-2003. with much higher prevalence among patients who are not treated early in the course of illness. Kawasaki disease and [[rheumatic fever]] are most common causes of acquired heart disease among children in the United States. ==Symptoms== Kawasaki disease often begins with a high and persistent [[fever]] that is not very responsive to normal doses of [[paracetamol]] (acetaminophen) or [[ibuprofen]]. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes, red [[mucous membrane]]s in the mouth, red cracked lips, a "[[strawberry tongue]]", iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen [[lymph node]]s. [[Skin rash]]es occur early in the disease, and peeling of the skin in the [[genital area]], hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to [[myocardial infarction]] (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short. * High-grade fever (greater than 39&nbsp;°C or 102&nbsp;°F; often as high as 40&nbsp;°C or 104&nbsp;°F) that normally lasts for more than a week if left untreated. * Red eyes ([[conjunctivitis]]) without pus or drainage, also known as "conjunctival injection" * Bright red, chapped, or cracked lips * Red [[mucous membrane]]s in the mouth * Strawberry tongue, white coating on the tongue or prominent red bumps ([[wiktionary:Papillae|papillae]]) on the back of the tongue * Red palms of the hands and the soles of the feet * Swollen hands and feet * Rash which may take many forms, but not vesicular (blister-like), on the trunk * Swollen [[lymph node]]s (frequently only one lymph node is swollen), particularly in the neck area * Joint pain ([[arthralgia]]) and swelling, frequently symmetrical * Irritability * [[Tachycardia]] (rapid heart beat) * Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails Beaus lines(transverse grooves on nails) ==Signs and tests== A physical examination will demonstrate many of the features listed above. Blood tests * [[Complete blood count]] (CBC) may reveal normocytic [[anemia]] and eventually [[thrombocytosis]] * [[Erythrocyte sedimentation rate]] (ESR) will be elevated * [[C-reactive protein]] (CRP) will be elevated * [[Liver function tests]] may show evidence of hepatic inflammation and low [[serum albumin]] Other tests (may or may not be performed) * [[Electrocardiogram]] may show evidence of [[ventricular]] dysfunction or, occasionally, [[arrhythmia]] due to [[myocarditis]] * [[Echocardiogram]] may show subtle coronary artery changes or, later, true aneurysms. * [[Ultrasound]] or [[computerized tomography]] may show hydrops (enlargement) of the [[gallbladder]] * [[Urinalysis]] may show white blood cells and protein in the urine ([[pyuria]] and [[proteinuria]]) without evidence of bacterial growth * [[Lumbar puncture]] may show evidence of [[aseptic meningitis]] * [[Angiography]] was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography. If not treated in time it can cause many heart problems. ==Diagnosis== Kawasaki disease can only be diagnosed clinically (by [[medical sign]]s and [[symptom]]s), as there exists no specific laboratory test that can tell if someone has it. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen their doctor several times, or visited a number of different health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the [[differential diagnosis]], including [[scarlet fever]], [[toxic shock]] syndrome, and [[juvenile idiopathic arthritis]]. Classically, five days of fever<ref name="urlKawasaki Disease - June 1999 - American Academy of Family Physicians">{{cite web |url=http://www.aafp.org/afp/990600ap/3093.html |title=Kawasaki Disease - June 1999 - American Academy of Family Physicians |format= |work= |accessdate=}}</ref> plus four of five [[diagnostic]] [[criteria]] must be met in order to establish the diagnosis. The criteria are: (1) [[erythema]] of the lips or oral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes (conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters. Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting. ==Treatment== Children with Kawasaki disease should be hospitalized and cared for by a physician who has experience with this disease. When in an academic medical center, care is often shared between pediatric [[cardiology]] and pediatric [[infectious disease]] specialists, although no [[infectious agent]] has been demonstrated. It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the [[coronary arteries]]. [[Intravenous immunoglobulin]] ([[IVIG]]) is the standard treatment for Kawasaki disease<ref name="pmid14584002">{{cite journal |author=Oates-Whitehead RM, Baumer JH, Haines L, ''et al'' |title=Intravenous immunoglobulin for the treatment of Kawasaki disease in children |journal=Cochrane Database Syst Rev |volume= |issue=4 |pages=CD004000 |year=2003 |pmid=14584002 |doi=10.1002/14651858.CD004000}}</ref> and is administered in high doses with marked improvement usually noted within 24 hours. If the fever does not respond, an additional dose may have to be considered. IVIG by itself is most useful within the first 7 days of onset of fever, in terms of preventing coronary artery aneurysm. [[Salicylate]] therapy, particularly [[aspirin]], remains an important part of the treatment (though questioned by some)<ref name="pmid15545617">{{cite journal |author=Hsieh KS, Weng KP, Lin CC, Huang TC, Lee CL, Huang SM |title=Treatment of acute Kawasaki disease: aspirin's role in the febrile stage revisited |journal=Pediatrics |volume=114 |issue=6 |pages=e689–93 |year=2004 |month=December |pmid=15545617 |doi=10.1542/peds.2004-1037 |url=http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=15545617}}</ref> but [[salicylate]]s alone are not as effective as [[Intravenous immunoglobulin]]. 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 2 months to prevent blood clots from forming. Except for Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended for children due to its association with [[Reye's syndrome]]. [[Corticosteroids]] have also been used,<ref name="pmid12838187">{{cite journal |author=Sundel RP, Baker AL, Fulton DR, Newburger JW |title=Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial |journal=J. Pediatr. |volume=142 |issue=6 |pages=611–6 |year=2003 |month=June |pmid=12838187 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0022347603001173}}</ref> especially when other treatments fail or symptoms recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin and aspirin did not improve outcome. <ref>Newburger JW et al, Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease, N Engl J Med. 2007 Feb 25;356(7):663-75</ref> There are also treatments for iritis and other eye symptoms. ==Prognosis== With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (''i.e.'' the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an [[echocardiogram]] initially every few weeks, and then every 1&ndash;2 years to screen for progression of cardiac involvement. It is also not uncommon that a [[relapse]] of symptoms may occur soon after initial treatment with [[IVIG]]. This usually requires re-hospitalization and retreatment. Treatment with [[IVIG]] can cause allergic and non-allergic acute reactions, aseptic meningitis, [[fluid overload]] and, rarely, other serious reactions. Aspirin may increase the risk of bleeding from other causes and may be associated with Reye's syndrome. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment. ==References== {{reflist|2}} ==External links== *[http://www.kdfoundation.org/ Kawasaki Disease Foundation] *[http://www.kdforum.org/ Kawasaki Disease Forum] *[http://www.kdcanada.ca/ Kawasaki Disease Canada] *[http://www.emory.edu/CHCS/p_histmed_Kawasaki.ht Kawasaki Disease Research Program] * [http://heart.seattlechildrens.org/conditions_treated/kawasaki_disease.asp Kawasaki Disease information] from Seattle Children's Hospital Heart Center * {{GPnotebook|1751842816}} {{Diseases of the musculoskeletal system and connective tissue}} [[Category:Cardiology]] [[Category:Pediatrics]] [[de:Kawasaki-Syndrom]] [[es:Enfermedad de Kawasaki]] [[fr:Maladie de Kawasaki]] [[it:Sindrome di Kawasaki]] [[he:מחלת קווסקי]] [[nl:Ziekte van Kawasaki]] [[ja:川崎病]] [[pl:Choroba Kawasaki]] [[pt:Doença de Kawasaki]] [[th:โรคคาวาซากิ]] [[zh:川崎氏病]]