Kawasaki disease: Clinical features and diagnosis
- Robert Sundel, MD
Robert Sundel, MD
- Section Editor — Pediatric Rheumatology
- Associate Professor of Pediatrics
- Harvard Medical School
- Section Editors
- Marisa Klein-Gitelman, MD, MPH
Marisa Klein-Gitelman, MD, MPH
- Section Editor — Pediatric Rheumatology
- Chief, Division of Rheumatology
- Ann & Robert H. Lurie Children's Hospital of Chicago
- Professor of Pediatrics
- Northwestern University Feinberg School of Medicine
- Sheldon L Kaplan, MD
Sheldon L Kaplan, MD
- Editor-in-Chief — Pediatrics
- Section Editor — Pediatric Infectious Diseases
- Professor and Vice Chairman for Clinical Affairs
- Baylor College of Medicine
Kawasaki disease (KD, previously called mucocutaneous lymph node syndrome) is one of the most common vasculitides of childhood . KD also occurs rarely in adults. It is typically a self-limited condition, with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy . However, complications such as coronary artery (CA) aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, arrhythmias, and peripheral arterial occlusion may develop and lead to significant morbidity and mortality. (See "Cardiovascular sequelae of Kawasaki disease".)
The clinical manifestations and diagnosis of KD are discussed in this review. The epidemiology, etiology, treatment, and complications of KD, including cardiac sequelae, are presented separately. Incomplete (atypical) KD and unique features in infants and adults are also reviewed separately. (See "Kawasaki disease: Epidemiology and etiology" and "Kawasaki disease: Initial treatment and prognosis" and "Cardiovascular sequelae of Kawasaki disease" and "Incomplete (atypical) Kawasaki disease" and "Kawasaki disease: Complications".)
The clinical features of KD reflect widespread inflammation of primarily medium-sized muscular arteries. Diagnosis is based upon evidence of systemic inflammation (eg, fever) in association with signs of mucocutaneous inflammation. The characteristic bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, rash, extremity changes, and cervical lymphadenopathy typically develop after a brief nonspecific prodrome of respiratory or gastrointestinal symptoms [3-8] (see 'Other findings' below). These characteristic clinical signs are the basis for the diagnostic criteria for KD (table 1) .
Oral mucous membrane findings are seen in approximately 90 percent of cases of KD, polymorphous rash in 70 to 90 percent, extremity changes in 50 to 85 percent, ocular changes in >75 percent, and cervical lymphadenopathy in 25 to 70 percent [7,10-12].
These findings are often not present at the same time, and there is no typical order of appearance. As an example, some patients have only developed fever and cervical lymphadenopathy by the time of admission (so-called KD with isolated cervical lymphadenopathy, KDiL) . In one case series, these patients tended to be older and to have a more severe course, with increased risk of coronary artery (CA) disease and lack of response to intravenous immune globulin (IVIG). Thus, repeated histories and physical examinations are important both for making a timely diagnosis of KD in children who fail to meet diagnostic criteria, as well as for appropriate consideration of alternative diagnoses. (See 'Diagnosis' below.)
- Burns JC, Glodé MP. Kawasaki syndrome. Lancet 2004; 364:533.
- Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 2004; 110:2747.
- Morens DM, Anderson LJ, Hurwitz ES. National surveillance of Kawasaki disease. Pediatrics 1980; 65:21.
- Huang GY, Ma XJ, Huang M, et al. Epidemiologic pictures of Kawasaki disease in Shanghai from 1998 through 2002. J Epidemiol 2006; 16:9.
- Baker AL, Lu M, Minich LL, et al. Associated symptoms in the ten days before diagnosis of Kawasaki disease. J Pediatr 2009; 154:592.
- Burns JC, Mason WH, Glode MP, et al. Clinical and epidemiologic characteristics of patients referred for evaluation of possible Kawasaki disease. United States Multicenter Kawasaki Disease Study Group. J Pediatr 1991; 118:680.
- Ozdemir H, Ciftçi E, Tapisiz A, et al. Clinical and epidemiological characteristics of children with Kawasaki disease in Turkey. J Trop Pediatr 2010; 56:260.
- Cai Z, Zuo R, Liu Y. Characteristics of Kawasaki disease in older children. Clin Pediatr (Phila) 2011; 50:952.
- Ayusawa M, Sonobe T, Uemura S, et al. Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition). Pediatr Int 2005; 47:232.
- Fukushige J, Takahashi N, Ueda Y, Ueda K. Incidence and clinical features of incomplete Kawasaki disease. Acta Paediatr 1994; 83:1057.
- Sung RY, Ng YM, Choi KC, et al. Lack of association of cervical lymphadenopathy and coronary artery complications in Kawasaki disease. Pediatr Infect Dis J 2006; 25:521.
- Germain BF, Moroney JD, Guggino GS, et al. Anterior uveitis in Kawasaki disease. J Pediatr 1980; 97:780.
- Nomura Y, Arata M, Koriyama C, et al. A severe form of Kawasaki disease presenting with only fever and cervical lymphadenopathy at admission. J Pediatr 2010; 156:786.
- Smith LB, Newburger JW, Burns JC. Kawasaki syndrome and the eye. Pediatr Infect Dis J 1989; 8:116.
- Eberhard BA, Sundel RP, Newburger JW, et al. Psoriatic eruption in Kawasaki disease. J Pediatr 2000; 137:578.
- Kishimoto S, Muneuchi J, Takahashi Y, et al. Psoriasiform skin lesion and supprative acrodermatitis associated with Kawasaki disease followed by the treatment with infliximab: a case report. Acta Paediatr 2010; 99:1102.
- Ergin S, Karaduman A, Demirkaya E, et al. Plaque psoriasis induced after Kawasaki disease. Turk J Pediatr 2009; 51:375.
- Liao YC, Lee JY. Psoriasis in a 3-month-old infant with Kawasaki disease. Dermatol Online J 2009; 15:10.
- Wang S, Best BM, Burns JC. Periungual desquamation in patients with Kawasaki disease. Pediatr Infect Dis J 2009; 28:538.
- April MM, Burns JC, Newburger JW, Healy GB. Kawasaki disease and cervical adenopathy. Arch Otolaryngol Head Neck Surg 1989; 115:512.
- Kanegaye JT, Van Cott E, Tremoulet AH, et al. Lymph-node-first presentation of Kawasaki disease compared with bacterial cervical adenitis and typical Kawasaki disease. J Pediatr 2013; 162:1259.
- Dominguez SR, Anderson MS, El-Adawy M, Glodé MP. Preventing coronary artery abnormalities: a need for earlier diagnosis and treatment of Kawasaki disease. Pediatr Infect Dis J 2012; 31:1217.
- Printz BF, Sleeper LA, Newburger JW, et al. Noncoronary cardiac abnormalities are associated with coronary artery dilation and with laboratory inflammatory markers in acute Kawasaki disease. J Am Coll Cardiol 2011; 57:86.
- Gong GW, McCrindle BW, Ching JC, Yeung RS. Arthritis presenting during the acute phase of Kawasaki disease. J Pediatr 2006; 148:800.
- Melish ME. Kawasaki syndrome: a 1986 perspective. Rheum Dis Clin North Am 1987; 13:7.
- McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation 2017; 135:e927.
- Nofech-Mozes Y, Garty BZ. Thrombocytopenia in Kawasaki disease: a risk factor for the development of coronary artery aneurysms. Pediatr Hematol Oncol 2003; 20:597.
- Shike H, Kanegaye JT, Best BM, et al. Pyuria associated with acute Kawasaki disease and fever from other causes. Pediatr Infect Dis J 2009; 28:440.
- Watanabe T, Abe Y, Sato S, et al. Sterile pyuria in patients with Kawasaki disease originates from both the urethra and the kidney. Pediatr Nephrol 2007; 22:987.
- Eladawy M, Dominguez SR, Anderson MS, Glodé MP. Abnormal liver panel in acute kawasaki disease. Pediatr Infect Dis J 2011; 30:141.
- Dengler LD, Capparelli EV, Bastian JF, et al. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J 1998; 17:478.
- Hicks RV, Melish ME. Kawasaki syndrome. Pediatr Clin North Am 1986; 33:1151.
- Salo E, Pesonen E, Viikari J. Serum cholesterol levels during and after Kawasaki disease. J Pediatr 1991; 119:557.
- Newburger JW, Burns JC, Beiser AS, Loscalzo J. Altered lipid profile after Kawasaki syndrome. Circulation 1991; 84:625.
- Cabana VG, Gidding SS, Getz GS, et al. Serum amyloid A and high density lipoprotein participate in the acute phase response of Kawasaki disease. Pediatr Res 1997; 42:651.
- Nakamura Y, Yashiro M, Uehara R, et al. Use of laboratory data to identify risk factors of giant coronary aneurysms due to Kawasaki disease. Pediatr Int 2004; 46:33.
- Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi 1967; 16:178.
- Centers for Disease Control. Kawasaki disease — New York. MMWR Morb Mortal Wkly Rep 1980; 29:61.
- Uehara R, Igarashi H, Yashiro M, et al. Kawasaki disease patients with redness or crust formation at the Bacille Calmette-Guérin inoculation site. Pediatr Infect Dis J 2010; 29:430.
- Sundel RP. Update on the treatment of Kawasaki disease in childhood. Curr Rheumatol Rep 2002; 4:474.
- Yanagihara R, Todd JK. Acute febrile mucocutaneous lymph node syndrome. Am J Dis Child 1980; 134:603.
- Barron KS. Kawasaki disease in children. Curr Opin Rheumatol 1998; 10:29.
- Turnier JL, Anderson MS, Heizer HR, et al. Concurrent Respiratory Viruses and Kawasaki Disease. Pediatrics 2015; 136:e609.
- Benseler SM, McCrindle BW, Silverman ED, et al. Infections and Kawasaki disease: implications for coronary artery outcome. Pediatrics 2005; 116:e760.
- Jaggi P, Kajon AE, Mejias A, et al. Human adenovirus infection in Kawasaki disease: a confounding bystander? Clin Infect Dis 2013; 56:58.
- Furusho K, Kamiya T, Nakano H, et al. High-dose intravenous gammaglobulin for Kawasaki disease. Lancet 1984; 2:1055.
- Newburger JW, Takahashi M, Burns JC, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med 1986; 315:341.
- Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 2004; 114:1708.
- Minich LL, Sleeper LA, Atz AM, et al. Delayed diagnosis of Kawasaki disease: what are the risk factors? Pediatrics 2007; 120:e1434.