eMedicine Specialties > Rheumatology > Miscellaneous Inflammatory Arthritis

Kawasaki Disease: Differential Diagnoses & Workup

Author: Paul R Ogershok, MD, Allergist, Allergy, Asthma, and Immunology Clinic, Southwest Regional Medical Center
Coauthor(s): Martin Weisse, MD, Program Director, Associate Professor, Department of Pediatrics, West Virginia University
Contributor Information and Disclosures

Updated: Aug 6, 2009

Differential Diagnoses

Acute Rheumatic Fever
Toxic Shock Syndrome

Other Problems to Be Considered

Scarlet fever
Staphylococcal scalded skin syndrome
Stevens-Johnson syndrome
Drug reaction
Juvenile rheumatoid arthritis
Measles
Mercury poisoning
Other viral exanthems

Workup

Laboratory Studies

  • CBC count
    • Usually, the WBC count is elevated, with a predominance of immature and mature granulocytes.
    • Normocytic anemia may occur.
    • Thrombocytosis usually develops during the second or third week of illness, with an average range of 700,000/µL.
    • Thrombocytopenia is associated with severe coronary artery disease and myocardial infarction.
  • Acute-phase reactants
    • Erythrocyte sedimentation rate, C-reactive protein levels, and alpha1-antitrypsin levels are elevated.
    • The serum complement level is normal or elevated.
  • Liver enzymes
    • Transaminase values are mildly elevated in 40% of affected patients.
    • Bilirubin values are elevated in 10% of affected patients.
  • Urinalysis: Mild-to-moderate pyuria of urethral origin and proteinuria may occur.
  • Cardiac enzyme levels (eg, creatine kinase [CK], creatine kinase myocardial band [CK-MB], cardiac troponin, lactate dehydrogenase [LD-1 >LD-2]) are elevated during a myocardial infarction.
  • ECG
    • Tachycardia, prolonged PR interval, ST-T wave changes, and decreased voltage of R waves may indicate myocarditis.
    • Q waves or ST-T wave changes may indicate myocardial infarction.

Imaging Studies

  • Obtain a chest radiograph to exclude cardiomegaly or subclinical pneumonitis.
  • Echocardiography
    • Perform at baseline to exclude coronary artery aneurysms and evidence of myocarditis, valvulitis, or pericardial effusion.
    • In children, ensure that a pediatric cardiologist performs this study because of familiarity with coronary artery diameters. Diffuse dilatation of coronary lumina can be observed in 50% of patients by the 10th day of illness.
  • Coronary CT angiography and magnetic resonance angiography may also prove beneficial in the evaluation and follow-up of the coronary arteries.4,5

More on Kawasaki Disease

Overview: Kawasaki Disease
Differential Diagnoses & Workup: Kawasaki Disease
Treatment & Medication: Kawasaki Disease
Follow-up: Kawasaki Disease
Multimedia: Kawasaki Disease
References
Further Reading
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References

  1. Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi. Mar 1967;16(3):178-222. [Medline].

  2. Uehara R, Yashiro M, Nakamura Y, Yanagawa H. Clinical features of patients with Kawasaki disease whose parents had the same disease. Arch Pediatr Adolesc Med. Dec 2004;158(12):1166-9. [Medline].

  3. Onouchi Y, Tamari M, Takahashi A, Tsunoda T, Yashiro M, Nakamura Y, et al. A genomewide linkage analysis of Kawasaki disease: evidence for linkage to chromosome 12. J Hum Genet. 2007;52(2):179-90. [Medline].

  4. Goo HW, Park IS, Ko JK, Kim YH. Coronary CT angiography and MR angiography of Kawasaki disease. Pediatr Radiol. Jul 2006;36(7):697-705. [Medline].

  5. Mavrogeni S, Papadopoulos G, Douskou M, Kaklis S, Seimenis I, Baras P, et al. Magnetic resonance angiography is equivalent to X-ray coronary angiography for the evaluation of coronary arteries in Kawasaki disease. J Am Coll Cardiol. Feb 18 2004;43(4):649-52. [Medline].

  6. [Best Evidence] Baumer JH, Love SJ, Gupta A, Haines LC, Maconochie I, Dua JS. Salicylate for the treatment of Kawasaki disease in children. Cochrane Database Syst Rev. 2006;(4):CD004175. [Medline].

  7. Hsieh KS, Weng KP, Lin CC, Huang TC, Lee CL, Huang SM. Treatment of acute Kawasaki disease: aspirin's role in the febrile stage revisited. Pediatrics. Dec 2004;114(6):e689-93. [Medline].

  8. [Best Evidence] Inour Y, Okada Y, Shinohara M, Kobayashi T, Kobayashi T, Tomomasa T, et al. A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome. J Pediatr. Sep/2006;149(3):336-341. [Medline].

  9. [Best Evidence] Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med. Feb/2007;356(7):663-75. [Medline].

  10. Anderson MS, Todd JK, Glode MP. Delayed diagnosis of Kawasaki syndrome: an analysis of the problem. Pediatrics. Apr 2005;115(4):e428-33. [Medline].

  11. Baker AL, Lu M, Minich LL, Atz AM, Klein GL, Korsin R, et al. Associated symptoms in the ten days before diagnosis of Kawasaki disease. J Pediatr. Apr 2009;154(4):592-595.e2. [Medline].

  12. Barron KS, Shulman ST, Rowley A, Taubert K, Myones BL, Meissner HC, et al. Report of the National Institutes of Health Workshop on Kawasaki Disease. J Rheumatol. Jan 1999;26(1):170-90. [Medline].

  13. Burns JC, Best BM, Mejias A, Mahony L, Fixler DE, Jafri HS, et al. Infliximab treatment of intravenous immunoglobulin-resistant Kawasaki disease. J Pediatr. Dec 2008;153(6):833-8. [Medline].

  14. Burns JC, Shike H, Gordon JB, Malhotra A, Schoenwetter M, Kawasaki T. Sequelae of Kawasaki disease in adolescents and young adults. J Am Coll Cardiol. Jul 1996;28(1):253-7. [Medline].

  15. Feigin RD, Cecchin F. Disorders of Unknown Etiology: Kawasaki Disease. In: Oski's Pediatrics Principles and Practice. 3rd ed. 1999:924-32.

  16. Friter BS, Lucky AW. The perineal eruption of Kawasaki syndrome. Arch Dermatol. Dec 1988;124(12):1805-10. [Medline].

  17. Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, et al. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation. Mar 24 2009;119(11):1541-51. [Medline].

  18. Harnden A, Takahashi M, Burgner D. Kawasaki disease. BMJ. May 5 2009;338:b1514. [Medline].

  19. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics. Sep 1974;54(3):271-6. [Medline].

  20. Kogulan P, Mbualungu E, Villanueva E. Kawasaki syndrome in an adult: case report and review of the literature in adolescents and adults. J Clin Rheumatol. 2001;7:194-8.

  21. Mason WH, Takahashi M. Kawasaki syndrome. Clin Infect Dis. Feb 1999;28(2):169-85; quiz 186-7. [Medline].

  22. Morens DM, Melish ME. Unclassified Infectious Diseases: Kawasaki Disease. In: Textbook of Pediatric Infectious Diseases. 4th ed. 1998:995-1014.

  23. Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC, 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. Dec 2004;114(6):1708-33. [Medline].

  24. Pahl E. Kawasaki disease: cardiac sequelae and management. Pediatr Ann. Feb 1997;26(2):112-5. [Medline].

  25. Rowley AH, Shulman ST. Kawasaki syndrome. Pediatr Clin North Am. Apr 1999;46(2):313-29. [Medline].

  26. Senzaki H. Long-term outcome of Kawasaki disease. Circulation. Dec 16 2008;118(25):2763-72. [Medline].

  27. [Guideline] Stapp J, Marshall GS. Fulfillment of diagnostic criteria in Kawasaki disease. South Med J. Jan 2000;93(1):44-7. [Medline].

  28. Taubert KA, Shulman ST. Kawasaki disease. Am Fam Physician. Jun 1999;59(11):3093-102, 3107-8. [Medline].

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Keywords

KD, Kawasaki syndrome, Kawasaki's syndrome, Kawasaki disease, Kawasaki's disease, mucocutaneous lymph node syndrome, infantile periarteritis nodosa, vasculitis, atypical KD, incomplete KD, atypical Kawasaki disease, incomplete Kawasaki disease, polymorphous rash, bilateral conjunctival injection, erythematous rash

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Contributor Information and Disclosures

Author

Paul R Ogershok, MD, Allergist, Allergy, Asthma, and Immunology Clinic, Southwest Regional Medical Center
Paul R Ogershok, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, Pennsylvania Medical Society, and West Virginia State Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Martin Weisse, MD, Program Director, Associate Professor, Department of Pediatrics, West Virginia University
Martin Weisse, MD is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Pediatric Infectious Diseases Society
Disclosure: Nothing to disclose.

Medical Editor

Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center
Lawrence H Brent, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, and American College of Rheumatology
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Grant/research funds Other; Amgen Honoraria Speaking and teaching; Wyeth Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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