Sections

Medication

Immunomodulatory agents

Class Summary

IVIG is a purified preparation of gamma globulin. It is derived from large pools of human plasma comprising 4 subclasses of antibodies, approximating the distribution of human serum.

These agents are used to improve clinical and immunologic aspects of Kawasaki disease. They may decrease autoantibody production and increase solubilization and removal of immune complexes.

Immune globulin, intravenous (Carimune, Gammagard, Gamunex-C, Octagam)

View full drug information

IVIG is generally recommended as first-line therapy. It neutralizes circulating myelin antibodies by means of anti-idiotypic antibodies; downregulates proinflammatory cytokines, including interferon-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; and promotes remyelination. It may increase cerebrospinal fluid IgG levels (10%).

IVIG reduces the prevalence of coronary abnormalities. It leads to rapid defervescence and more rapid normalization of acute-phase reactants.

Class Summary

These agents inhibit prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2. Adequate anti-inflammatory therapy requires that aspirin be combined with gamma globulin. Children with coronary artery disease have received aspirin for prolonged periods.

Aspirin (Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin, Ecotrin)

View full drug information

Aspirin is used to decrease inflammation, inhibit platelet aggregation, and improve complications of venous stasis and thrombosis. It irreversibly inactivates cyclooxygenase, ultimately preventing thromboxane A2 production in platelets. Platelet function does not fully recover until new platelets are made (in 7-10 days). It is first-line therapy, along with IVIG.

Oral absorption may decrease in Kawasaki disease to < 50% (vs typical bioavailability of 85-90%). Altered bioavailability may explain why higher doses are required to achieve a salicylate serum concentration >20 mg/dL.

Class Summary

Besides aspirin, dipyridamole may be used to prevent microthrombus formation.

Dipyridamole (Persantine)

View full drug information

Dipyridamole is a platelet-adhesion inhibitor that possibly inhibits red blood cell uptake of adenosine, itself an inhibitor of platelet reactivity. It may inhibit phosphodiesterase activity, leading to increased cyclic adenosine monophosphate (cAMP) levels in platelets and formation of potent platelet activator thromboxane A2.

Class Summary

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisolone acetate (Prelone, Flo-Pred, Millipred)

View full drug information

Prednisolone is indicated for the treatment of steroid-responsive inflammation of the palpebral and bulbar conjunctiva, cornea, and anterior segment. It decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

Methylprednisolone (A-Methapred, Medrol, Solu-Medrol)

View full drug information

Methylprednisolone decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

Class Summary

These agents inhibit key factors that mediate immune reactions.

Infliximab (Remicade)

View full drug information

Infliximab may be added to treatment if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. Infliximab is a chimeric IgG1k monoclonal antibody that neutralizes cytokine TNF-α and inhibits its binding to the TNF-α receptor. It reduces the infiltration of inflammatory cells and TNF-α production in inflamed areas.

Clopidogrel (Plavix)

View full drug information

Questions

Son MBF, Newburger JW. Kawasaki Disease. Pediatr Rev. 2018 Feb. 39 (2):78-90. [QxMD MEDLINE Link].
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 Apr 25. 135 (17):e927-e999. [QxMD MEDLINE Link].
Satou GM, Giamelli J, Gewitz MH. Kawasaki disease: diagnosis, management, and long-term implications. Cardiol Rev. 2007 Jul-Aug. 15(4):163-9. [QxMD MEDLINE Link].
Wood L, Tulloh R. Kawasaki disease: diagnosis, management and cardiac sequelae. Expert Rev Cardiovasc Ther. 2007 May. 5(3):553-61. [QxMD MEDLINE Link].
Newburger JW, Takahashi M, Burns JC, Beiser AS, Chung KJ, Duffy CE, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med. 1986 Aug 7. 315(6):341-7. [QxMD MEDLINE Link].
Kawamura Y, Kanai T, Takeshita S, Nonoyama S. Use of the urinary trypsin inhibitor ulinastatin for acute Kawasaki disease. Nihon Rinsho. 2014 Sep. 72(9):1650-3. [QxMD MEDLINE Link].
Kawasaki T. [Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]. Arerugi. 1967 Mar. 16(3):178-222. [QxMD MEDLINE Link].
Melish ME, Hicks RM, Larson EJ. Mucocutaneous lymph node syndrome in the United States. Am J Dis Child. 1976 Jun. 130(6):599-607. [QxMD MEDLINE Link].
Rowley AH, Shulman ST. Pathogenesis and management of Kawasaki disease. Expert Rev Anti Infect Ther. 2010 Feb. 8(2):197-203. [QxMD MEDLINE Link]. [Full Text].
Burns JC, Shimizu C, Shike H, Newburger JW, Sundel RP, Baker AL, et al. Family-based association analysis implicates IL-4 in susceptibility to Kawasaki disease. Genes Immun. 2005 Aug. 6(5):438-44. [QxMD MEDLINE Link]. [Full Text].
Lee TJ, Chun JK, Yeon SI, Shin JS, Kim DS. Increased serum levels of macrophage migration inhibitory factor in patients with Kawasaki disease. Scand J Rheumatol. 2007 May-Jun. 36(3):222-5. [QxMD MEDLINE Link].
Leung DY, Schlievert PM, Meissner HC. The immunopathogenesis and management of Kawasaki syndrome. Arthritis Rheum. 1998 Sep. 41(9):1538-47. [QxMD MEDLINE Link].
Pietra BA, De Inocencio J, Giannini EH, Hirsch R. TCR V beta family repertoire and T cell activation markers in Kawasaki disease. J Immunol. 1994 Aug 15. 153(4):1881-8. [QxMD MEDLINE Link].
Wang CL, Wu YT, Liu CA, Kuo HC, Yang KD. Kawasaki disease: infection, immunity and genetics. Pediatr Infect Dis J. 2005 Nov. 24(11):998-1004. [QxMD MEDLINE Link].
Onouchi Y. Susceptibility genes for Kawasaki disease. Nihon Rinsho. 2014 Sep. 72(9):1554-60. [QxMD MEDLINE Link].
Treadwell TA, Maddox RA, Holman RC, Belay ED, Shahriari A, Anderson MS, et al. Investigation of Kawasaki syndrome risk factors in Colorado. Pediatr Infect Dis J. 2002 Oct. 21(10):976-8. [QxMD MEDLINE Link].
Burns JC, Glodé MP. Kawasaki syndrome. Lancet. 2004 Aug 7-13. 364(9433):533-44. [QxMD MEDLINE Link].
Melish ME, Hicks RV. Kawasaki syndrome: clinical features. Pathophysiology, etiology and therapy. J Rheumatol Suppl. 1990 Sep. 24:2-10. [QxMD MEDLINE Link].
Leung DY, Meissner HC, Fulton DR, Murray DL, Kotzin BL, Schlievert PM. Toxic shock syndrome toxin-secreting Staphylococcus aureus in Kawasaki syndrome. Lancet. 1993 Dec 4. 342(8884):1385-8. [QxMD MEDLINE Link].
Yanagawa H, Nakamura Y, Yashiro M, Ojima T, Tanihara S, Oki I, et al. Results of the nationwide epidemiologic survey of Kawasaki disease in 1995 and 1996 in Japan. Pediatrics. 1998 Dec. 102(6):E65. [QxMD MEDLINE Link].
Yanagawa H, Yashiro M, Nakamura Y, Kawasaki T, Kato H. Epidemiologic pictures of Kawasaki disease in Japan: from the nationwide incidence survey in 1991 and 1992. Pediatrics. 1995 Apr. 95(4):475-9. [QxMD MEDLINE Link].
Pinna GS, Kafetzis DA, Tselkas OI, Skevaki CL. Kawasaki disease: an overview. Curr Opin Infect Dis. 2008 Jun. 21(3):263-70. [QxMD MEDLINE Link].
Caquard M, Parlier G, Siret D. [Family observation of Kawasaki disease: 2 cases in sister and brother]. Arch Pediatr. 2006 May. 13(5):453-5. [QxMD MEDLINE Link].
Burns JC, Shimizu C, Gonzalez E, Kulkarni H, Patel S, Shike H, et al. Genetic variations in the receptor-ligand pair CCR5 and CCL3L1 are important determinants of susceptibility to Kawasaki disease. J Infect Dis. 2005 Jul 15. 192(2):344-9. [QxMD MEDLINE Link]. [Full Text].
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. 2004 Dec. 158(12):1166-9. [QxMD MEDLINE Link].
Mason WH, Takahashi M, Schneider T. Recurrence of Kawasaki disease in a large urban cohort in the United States. In: Takahashi M, Taubert K, eds. Proceedings of the Fourth International Symposium on Kawasaki Disease. Dallas, Tex: American Heart Association; 1993:21-6.:
Kato S, Kimura M, Tsuji K, Kusakawa S, Asai T, Juji T, et al. HLA antigens in Kawasaki disease. Pediatrics. 1978 Feb. 61(2):252-5. [QxMD MEDLINE Link].
Matsuda I, Hattori S, Nagata N, Fruse A, Nambu H. HLA antigens in mucocutaneous lymph node syndrome. Am J Dis Child. 1977 Dec. 131(12):1417-8. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Dergun M, Kao A, Hauger SB, Newburger JW, Burns JC. Familial occurrence of Kawasaki syndrome in North America. Arch Pediatr Adolesc Med. 2005 Sep. 159(9):876-81. [QxMD MEDLINE Link]. [Full Text].
Newburger JW, Taubert KA, Shulman ST, Rowley AH, Gewitz MH, Takahashi M, et al. Summary and abstracts of the Seventh International Kawasaki Disease Symposium: December 4-7, 2001, Hakone, Japan. Pediatr Res. 2003 Jan. 53(1):153-7. [QxMD MEDLINE Link].
Burns JC, Cayan DR, Tong G, Bainto EV, Turner CL, Shike H, et al. Seasonality and temporal clustering of Kawasaki syndrome. Epidemiology. 2005 Mar. 16(2):220-5. [QxMD MEDLINE Link]. [Full Text].
Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M, et al. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. Nat Genet. 2008 Jan. 40(1):35-42. [QxMD MEDLINE Link]. [Full Text].
Breunis WB, Biezeveld MH, Geissler J, Kuipers IM, Lam J, Ottenkamp J, et al. Polymorphisms in chemokine receptor genes and susceptibility to Kawasaki disease. Clin Exp Immunol. 2007 Oct. 150(1):83-90. [QxMD MEDLINE Link]. [Full Text].
Mamtani M, Matsubara T, Shimizu C, et al. Association of CCR2-CCR5 haplotypes and CCL3L1 copy number with Kawasaki Disease, coronary artery lesions, and IVIG responses in Japanese children. PLoS One. Jul 7 2010;5(7):e11458:
Taniuchi S, Masuda M, Teraguchi M, Ikemoto Y, Komiyama Y, Takahashi H, et al. Polymorphism of Fc gamma RIIa may affect the efficacy of gamma-globulin therapy in Kawasaki disease. J Clin Immunol. 2005 Jul. 25(4):309-13. [QxMD MEDLINE Link].
Holman RC, Belay ED, Christensen KY, Folkema AM, Steiner CA, Schonberger LB. Hospitalizations for Kawasaki syndrome among children in the United States, 1997-2007. Pediatr Infect Dis J. 2010 Jun. 29(6):483-8. [QxMD MEDLINE Link].
Makino N, Nakamura Y, Yashiro M, Ae R, Tsuboi S, Aoyama Y, et al. Descriptive epidemiology of Kawasaki disease in Japan, 2011-2012: from the results of the 22nd nationwide survey. J Epidemiol. 2015. 25 (3):239-45. [QxMD MEDLINE Link].
Burns JC, Mason WH, Hauger SB, Janai H, Bastian JF, Wohrley JD, et al. Infliximab treatment for refractory Kawasaki syndrome. J Pediatr. 2005 May. 146(5):662-7. [QxMD MEDLINE Link].
Park YW, Han JW, Park IS, Kim CH, Cha SH, Ma JS, et al. Kawasaki disease in Korea, 2003-2005. Pediatr Infect Dis J. 2007 Sep. 26(9):821-3. [QxMD MEDLINE Link].
Huang GY, Ma XJ, Huang M, Chen SB, Huang MR, Gui YH, et al. Epidemiologic pictures of Kawasaki disease in Shanghai from 1998 through 2002. J Epidemiol. 2006 Jan. 16(1):9-14. [QxMD MEDLINE Link].
Royle JA, Williams K, Elliott E, Sholler G, Nolan T, Allen R, et al. Kawasaki disease in Australia, 1993-95. Arch Dis Child. 1998 Jan. 78(1):33-9. [QxMD MEDLINE Link]. [Full Text].
Harnden A, Alves B, Sheikh A. Rising incidence of Kawasaki disease in England: analysis of hospital admission data. BMJ. 2002 Jun 15. 324(7351):1424-5. [QxMD MEDLINE Link]. [Full Text].
Lin YT, Manlhiot C, Ching JC, Han RK, Nield LE, Dillenburg R, et al. Repeated systematic surveillance of Kawasaki disease in Ontario from 1995 to 2006. Pediatr Int. 2010 Oct. 52(5):699-706. [QxMD MEDLINE Link].
Nakamura Y. Epidemiology of Kawasaki disease. Nihon Rinsho. 2014 Sep. 72(9):1536-41. [QxMD MEDLINE Link].
Wolff AE, Hansen KE, Zakowski L. Acute Kawasaki disease: not just for kids. J Gen Intern Med. 2007 May. 22(5):681-4. [QxMD MEDLINE Link]. [Full Text].
Stankovic K, Miailhes P, Bessis D, Ferry T, Broussolle C, Sève P. Kawasaki-like syndromes in HIV-infected adults. J Infect. 2007 Dec. 55(6):488-94. [QxMD MEDLINE Link].
Manlhiot C, Yeung RS, Clarizia NA, Chahal N, McCrindle BW. Kawasaki disease at the extremes of the age spectrum. Pediatrics. 2009 Sep. 124(3):e410-5. [QxMD MEDLINE Link].
Gedalia A. Kawasaki disease: 40 years after the original report. Curr Rheumatol Rep. 2007 Aug. 9(4):336-41. [QxMD MEDLINE Link].
Kim T, Choi W, Woo CW, Choi B, Lee J, Lee K, et al. Predictive risk factors for coronary artery abnormalities in Kawasaki disease. Eur J Pediatr. 2007 May. 166(5):421-5. [QxMD MEDLINE Link].
Yeo Y, Kim T, Ha K, Jang G, Lee J, Lee K, et al. Incomplete Kawasaki disease in patients younger than 1 year of age: a possible inherent risk factor. Eur J Pediatr. 2009 Feb. 168(2):157-62. [QxMD MEDLINE Link].
Kobayashi T, Inoue Y, Takeuchi K, Okada Y, Tamura K, Tomomasa T, et al. Prediction of intravenous immunoglobulin unresponsiveness in patients with Kawasaki disease. Circulation. 2006 Jun 6. 113 (22):2606-12. [QxMD MEDLINE Link].
Gupta-Malhotra M, Gruber D, Abraham SS, Roman MJ, Zabriskie JB, Hudgins LC, et al. Atherosclerosis in survivors of Kawasaki disease. J Pediatr. 2009 Oct. 155(4):572-7. [QxMD MEDLINE Link].
Kitamura S, Tsuda E, Kobayashi J, Nakajima H, Yoshikawa Y, Yagihara T, et al. Twenty-five-year outcome of pediatric coronary artery bypass surgery for Kawasaki disease. Circulation. 2009 Jul 7. 120(1):60-8. [QxMD MEDLINE Link].
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. 2009 Apr. 154(4):592-595.e2. [QxMD MEDLINE Link]. [Full Text].
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. Circulation. 2004 Oct 26. 110(17):2747-71. [QxMD MEDLINE Link].
Han RK, Sinclair B, Newman A, Silverman ED, Taylor GW, Walsh P, et al. Recognition and management of Kawasaki disease. CMAJ. 2000 Mar 21. 162(6):807-12. [QxMD MEDLINE Link]. [Full Text].
Turnier JL, Anderson MS, Heizer HR, Jone PN, Glodé MP, Dominguez SR. Concurrent Respiratory Viruses and Kawasaki Disease. Pediatrics. 2015 Aug 24. [QxMD MEDLINE Link].
Boggs W. Concurrent Respiratory Viruses Common in Children With Kawasaki Disease. http://www.medscape.com/viewarticle/850157. Available at http://www.medscape.com/viewarticle/850157. August 31, 2015; Accessed: September 14, 2015.
Henderson D. Kawasaki Disease Diagnosed by Urine Proteins? Medscape Medical News. December 26, 2012. Available at http://www.medscape.com/viewarticle/776728. Accessed: January 8, 2013.
Kentsis A, Shulman A, Ahmed S, Brennan E, Monuteaux MC et al. Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease. EMBO Mol Med. 2012 Dec 20. [QxMD MEDLINE Link].
Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB. Kawasaki syndrome and risk factors for coronary artery abnormalities: United States, 1994-2003. Pediatr Infect Dis J. 2006 Mar. 25(3):245-9. [QxMD MEDLINE Link].
Goo HW, Park IS, Ko JK, Kim YH. Coronary CT angiography and MR angiography of Kawasaki disease. Pediatr Radiol. 2006 Jul. 36(7):697-705. [QxMD MEDLINE Link].
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. 2004 Feb 18. 43(4):649-52. [QxMD MEDLINE Link].
Newburger JW, Fulton DR. Kawasaki disease. Curr Opin Pediatr. 2004 Oct. 16(5):508-14. [QxMD MEDLINE Link].
Hinze CH, Graham TB, Sutherell JS. Kawasaki disease without fever. Pediatr Infect Dis J. 2009 Oct. 28(10):927-8. [QxMD MEDLINE Link].
Printz BF, Sleeper LA, Newburger JW, Minich LL, Bradley T, Cohen MS, 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 Jan 4. 57(1):86-92. [QxMD MEDLINE Link].
Weedon R. Kawasaki Syndrome: The vasculopathic reaction pattern In:. Pathology. 2002:238-9:
Uehara R, Yashiro M, Oki I, Nakamura Y, Yanagawa H. Re-treatment regimens for acute stage of Kawasaki disease patients who failed to respond to initial intravenous immunoglobulin therapy: analysis from the 17th nationwide survey. Pediatr Int. 2007 Aug. 49(4):427-30. [QxMD MEDLINE Link].
Tremoulet AH, Best BM, Song S, Wang S, Corinaldesi E, Eichenfield JR, et al. Resistance to intravenous immunoglobulin in children with Kawasaki disease. J Pediatr. 2008 Jul. 153(1):117-21. [QxMD MEDLINE Link]. [Full Text].
Sittiwangkul R, Pongprot Y, Silvilairat S, Phornphutkul C. Management and outcome of intravenous gammaglobulin-resistant Kawasaki disease. Singapore Med J. 2006 Sep. 47(9):780-4. [QxMD MEDLINE Link].
Zulian F, Zanon G, Martini G, Mescoli G, Milanesi O. Efficacy of infliximab in long-lasting refractory Kawasaki disease. Clin Exp Rheumatol. 2006 Jul-Aug. 24(4):453. [QxMD MEDLINE Link].
Stenbøg EV, Windelborg B, Hørlyck A, Herlin T. The effect of TNFalpha blockade in complicated, refractory Kawasaki disease. Scand J Rheumatol. 2006 Jul-Aug. 35(4):318-21. [QxMD MEDLINE Link].
Burns JC, Best BM, Mejias A, Mahony L, Fixler DE, Jafri HS, et al. Infliximab treatment of intravenous immunoglobulin-resistant Kawasaki disease. J Pediatr. 2008 Dec. 153(6):833-8. [QxMD MEDLINE Link]. [Full Text].
Youn Y, Kim J, Hong YM, Sohn S. Infliximab as the First Retreatment in Patients with Kawasaki Disease Resistant to Initial Intravenous Immunoglobulin. Pediatr Infect Dis J. 2015 Dec 15. [QxMD MEDLINE Link].
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. 2004 Dec. 114(6):e689-93. [QxMD MEDLINE Link].
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 Oct 18. CD004175. [QxMD MEDLINE Link].
Inoue 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. 2006 Sep. 149(3):336-341. [QxMD MEDLINE Link].
Kobayashi T, Saji T, Otani T, Takeuchi K, Nakamura T, Arakawa H, et al. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial. Lancet. 2012 Apr 28. 379 (9826):1613-20. [QxMD MEDLINE Link].
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. 2007 Feb 15. 356(7):663-75. [QxMD MEDLINE Link].
Athappan G, Gale S, Ponniah T. Corticosteroid therapy for primary treatment of Kawasaki disease - weight of evidence: a meta-analysis and systematic review of the literature. Cardiovasc J Afr. 2009 Jul-Aug. 20(4):233-6. [QxMD MEDLINE Link].
Chen S, Dong Y, Kiuchi MG, Wang J, Li R, Ling Z, et al. Coronary Artery Complication in Kawasaki Disease and the Importance of Early Intervention : A Systematic Review and Meta-analysis. JAMA Pediatr. 2016 Oct 17. [QxMD MEDLINE Link].
Swift D. Kawasaki Disease: Add Corticosteroids to Initial IVIG. Medscape Medical News. Available at http://www.medscape.com/viewarticle/870767. October 21, 2016; Accessed: October 25, 2016.
[Guideline] Gorelik M, Chung SA, Ardalan K, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease. Arthritis Rheumatol. 2022 Apr. 74 (4):586-96. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Kawasaki disease: Patchy generalized macular erythema, which is also typical of some viral exanthems.
Kawasaki disease: Peeling and erythema of the fingertips.
Kawasaki disease: Strawberry tongue.
Pediatrics, Kawasaki disease. Note the appearance of the hand and lips. Photo courtesy of Sam Richardson, MD.
Clinical manifestations and time course of Kawasaki disease.
Oral manifestations of Kawasaki disease: red lips and strawberry tongue.
Video overview of Kawasaki disease pathophysiology, symptoms, diagnosis, and treatment.

of 7

Author

Tina K Sosa, MD Fellow in Pediatric Hospital Medicine, Cincinnati Children’s Hospital Medical Center

Tina K Sosa, MD is a member of the following medical societies: American Academy of Pediatrics, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Samir S Shah, MD, MSCE Professor, Department of Pediatrics, University of Cincinnati College of Medicine; Director, Division of Hospital Medicine, James M Ewell Endowed Chair, Attending Physician in Hospital Medicine and Infectious Diseases, Chief Metrics Officer, Cincinnati Children's Hospital Medical Center

Samir S Shah, MD, MSCE is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Pediatric Society, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, Society for Pediatric Research, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Chief Editor

Russell W Steele, MD Clinical Professor, Tulane University School of Medicine; Staff Physician, Ochsner Clinic Foundation

Russell W Steele, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric Infectious Diseases Society, Society for Pediatric Research, Southern Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Steven J Parrillo, DO, FACOEP, FACEP Clinical Adjunct Professor, Medical Director and Faculty, Disaster Medicine and Management Masters Program, Philadelphia University College of Health Sciences; Associate Professor, Clinical and Educational Scholarship Track, Jefferson Medical College of Thomas Jefferson University; Director, Division of EMS and Disaster Medicine, Albert Einstein Healthcare Network

Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, World Association for Disaster and Emergency Medicine

Disclosure: Nothing to disclose.

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, West Virginia State Medical Association

Disclosure: Nothing to disclose.

Noah S Scheinfeld, JD, MD, FAAD † Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Elena L Jones, MD Clinical Assistant Professor of Dermatology, Columbia University College of Physicians and Surgeons; Clinic Chief, Department of Dermatology, St Luke's-Roosevelt Hospital Center

Disclosure: Nothing to disclose.

Acknowledgements

Jeffrey Glenn Bowman, MD, MS Consulting Staff, Highfield MRI, Columbus, Ohio

Disclosure: Nothing to disclose.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of 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 for the Advancement of Science, 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; Pfizer Honoraria Speaking and teaching; Abbott Immunology Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; UCB Speaking and teaching; Omnicare Consulting fee Consulting; Centocor Consulting fee Consulting

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: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Joseph Domachowske, MD Professor of Pediatrics, Microbiology and Immunology, Department of Pediatrics, Division of Infectious Diseases, State University of New York Upstate Medical University

Joseph Domachowske, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

Catherine V Parrillo, DO, FACOP, FAAP, Retired, Clinical Assistant Professor, Department of Pediatrics, Philadelphia College of Osteopathic Medicine

Catherine V Parrillo, DO, FACOP, FAAP, is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Grace M Young, MD Associate Professor, Department of Pediatrics, University of Maryland Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians

Disclosure: Nothing to disclose.

Kawasaki Disease Medication

Immunomodulatory agents

Class Summary

Immune globulin, intravenous (Carimune, Gammagard, Gamunex-C, Octagam)

Immune globulin, intravenous (Carimune, Gammagard, Gamunex-C, Octagam)

Nonsteroidal Anti-Inflammatory Agents/Salicylates (NSAIDs)

Class Summary

Aspirin (Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin, Ecotrin)

Aspirin (Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin, Ecotrin)

Antiplatelet Agents, Hematologic