Kawasaki disease; mucocutaneous lymph node disease
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Introduction
Acute, febrile multisystem disease of children.
Etiology
- immune-mediated injury to endothelium
- immune reconstitution inflammatory syndrome in newly treated HIV1 patients
- association with Staphylococcus aureus
Epidemiology
- infants & children < 8 years of age
- may occur in adults, especially those with HIV1
Pathology
- systemic vasculitis of medium-sized to small arteries
Genetics
- associated with defects in ITPKC
Clinical manifestations
- acute febrile illness (high-spiking fevers)
- unresponsiveness to antibiotics
- non-suppurative cervical adenitis (50-75%)[4]
- mucosal membrane (mucositis)
- conjunctival congestion, conjunctivitis (90%)[12]
- erythema of oral cavity ("strawberry tongue")[8]
- erythema & fissuring of the lips
- anterior uveitis (70%)[12]
- pharyngitis
- skin
- edema of hands & feet
- scalatiniform rash or erythema multiforme with palmar erythema
- brawny induration of the extremities followed by desquamation
- desquamation of the skin of the fingertips
- perianal erythema[12]
- whole-body rash including oral mucositis[15]
- cardiac
- fever may pass & patients may present as adults with coronary artery vasculitis (see complications below)[15]
Laboratory
- complete blood count:
- leukocytosis (case report)[8]
- platelets normal (case report)[8]
- erythrocyte sedimentation rate elevated (case report)[8]
Radiology
- echocardiography at 1-2 weeks, if normal no repeat needed at 4-6 weeks
- stress echocardiography
- especially in patients with large coronary artery aneurysms, regardless of symptoms
- long-term surveillance required (see management - follow-up)
- especially in patients with large coronary artery aneurysms, regardless of symptoms
- coronary computed tomography angiography (see management - follow-up)
Complications
- mortality 0.5-2.8%
- vasculitis of coronary arteries in nearly all autopsies
- coronary artery aneurysms (most serious complication)
- coronary artery thrombosis[5][15] Differential diagnsosis:
- Multisystem inflammatory syndrome in children (MIS-C)[16]
Management
- uneventful recovery is usual
- pharmacologic therapy
- high dose intravenous gamma globulin (IVIG)
- 2 g/kg over 10 hours
- low-dose aspirin should be prescribed[16]
- forerly aspirin 100 mg/kg QD for 14 days followed by 3-5 mg/kg for several weeks; (high-dose salicylates)[5]
- synergistic with IVIG[12]
- a + b reduces incidence of coronary artery complications
- addition of glucocorticoids
- early adjunctive glucocorticoid therapy reduces fever duration & injury from coronary artery vasculitis[10]
- controversial[3]
- treatment for patients with IVIG resistance includes etanercept, cyclosporine, or infliximab[16]
- combined antiplatelet & direct oral anticoagulant therapy in patients with large coronary artery aneurysms[16]
- high dose intravenous gamma globulin (IVIG)
- follow-up
- patients with small aneurysms that have regressed with normal stress echocardiography & coronary computed tomography angiography do not require lifelong follow-up.
- patients with large coronary artery aneurysms require long-term surveillance
- children with Kawasaki disease require transitional care plans as they become adults
- some adults patients with Kawasaki disease in childhood do not require long-term cardiology follow-up.[16]
More general terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1678
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 93, 1920
- ↑ Jump up to: 3.0 3.1 Wooditch AC, Aronoff SC. Effect of initial corticosteroid therapy on coronary artery aneurysm formation in Kawasaki disease: a meta-analysis of 862 children. Pediatrics. 2005 Oct;116(4):989-95. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16199713
- ↑ Jump up to: 4.0 4.1 Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19501230
- ↑ Jump up to: 5.0 5.1 5.2 5.3 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2022.
- ↑ Seve P, Stankovic K, Smail A et al Adult Kawasaki disease: report of two cases and literature review. Semin Arthritis Rheum. 2005 Jun;34(6):785-92. PMID: https://www.ncbi.nlm.nih.gov/pubmed/15942913
- ↑ Gomard-Mennesson E, Landron C, Dauphin C et al Kawasaki disease in adults: report of 10 cases. Medicine (Baltimore). 2010 May;89(3):149-58 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20453601
- ↑ Jump up to: 8.0 8.1 8.2 8.3 8.4 Kole A, Chandakole D IMAGES IN CLINICAL MEDICINE. Strawberry Tongue. N Engl J Med 2015; 373:467. July 30, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/26222562 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1411222
- ↑ Scuccimarri R Kawasaki disease. Pediatr Clin North Am. 2012 Apr;59(2):425-45 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22560578
- ↑ Jump up to: 10.0 10.1 Chen S, Dong Y, Kiuchi MG et al Coronary Artery Complication in Kawasaki Disease and the Importance of Early Intervention A Systematic Review and Meta-analysis. JAMA Pediatrics. Oct 17, 2016 PMID: https://www.ncbi.nlm.nih.gov/pubmed/27749951
Sundel R. Clarifying the Role of Corticosteroids in Kawasaki Disease. JAMA Pediatr. 2016 Oct 17 PMID: https://www.ncbi.nlm.nih.gov/pubmed/27749946 - ↑ Cohen E, Sundel R. Kawasaki Disease at 50 Years. JAMA Pediatr. 2016 Sep 26. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27668809
- ↑ Jump up to: 12.0 12.1 12.2 12.3 12.4 12.5 12.6 Rashid RM, Barros BS (image) Hidden Heart Disease: 19 Dermatologic Clues You Should Know. Medscape. June 13, 2017. http://reference.medscape.com/slideshow/hidden-heart-disease-6004452
- ↑ Alikhan M, Lohr KM (images) Kawasaki Disease: Do You Know the Signs? Medscape. Sept 27, 2017 http://reference.medscape.com/slideshow/kawasaki-disease-6005698
- ↑ 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. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28356445
- ↑ Jump up to: 15.0 15.1 15.2 15.3 15.4 NEJM Knowledge+
Sundel RP Kawasaki disease Rheum Dis Clin North Am. 2015;41(1):63-73 PMID: https://www.ncbi.nlm.nih.gov/pubmed/25399940
Mirza SJ, Mohammed KO, Baslaib FO. Myocardial infarction in a young man due to coronary artery aneurysms after an undiagnosed Kawasaki disease. J Emerg Med. 2014 Jun;46(6):763-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24565879 - ↑ Jump up to: 16.0 16.1 16.2 16.3 Jone PN et al. Update on diagnosis and management of Kawasaki disease: A scientific statement from the American Heart Association. Circulation 2024 Dec 3; 150:e481 PMID: https://www.ncbi.nlm.nih.gov/pubmed/39534969 Free article https://www.ahajournals.org/doi/10.1161/CIR.0000000000001295
Patient information
Kawasaki disease patient information