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Kawasaki disease; mucocutaneous lymph node disease

Acute, febrile multisystem disease of children. Etiology: 1) immune-mediated injury to endothelium - immune reconstitution inflammatory syndrome in newly treated HIV1 patients 2) association with Staphylococcus aureus - toxic shock syndrome-1 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: 1) acute febrile illness (high-spiking fevers) 2) unresponsiveness to antibiotics 3) non-suppurative cervical adenitis (50-75%) [4] 4) mucosal membrane (mucositis) a) conjunctival congestion, conjunctivitis (90%) [12] b) erythema of oral cavity ("strawberry tongue") [8] c) erythema & fissuring of the lips d) anterior uveitis (70%) [12] e) pharyngitis 5) skin a) edema of hands & feet b) scalatiniform rash or erythema multiforme with palmar erythema c) brawny induration of the extremities followed by desquamation d) desquamation of the skin of the fingertips e) perianal erythema [12] f) whole-body rash including oral mucositis [15] 6) cardiac a) coronary artery vasculitis b) coronary artery aneurysm (25%) c) pericarditis d) myocarditis e) myocardial ischemia & infarction f) coronary artery thrombosis 7) 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] Complications: 1) mortality 0.5-2.8% 2) vasculitis of coronary arteries in nearly all autopsies - coronary artery aneurysms (most serious complication) - occur most often in subacute phase (when fever begins to subside) [12] - 25% of untreated children vs 1-5% of IVIG treated patients [12] - adults with childhood Kawasaki disease may have residual coronary artery aneurysms [5,15] - coronary artery thrombosis [5,15] Management: 1) uneventful recovery is usual 2) pharmacologic therapy a) high dose intravenous gamma globulin (IVIG) - 2 g/kg over 10 hours b) 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] c) a + b reduces incidence of coronary artery complications d) addition of glucocorticoids - early adjunctive glucocorticoid therapy reduces fever duration & injury from coronary artery vasculitis [10] - controversial [3]

Interactions

disease interactions

General

autoimmune disease vasculitis

Properties

PATHOLOGY: vascular aneurysm SITE: coronary artery ORIGIN: aortic sinus vasculitis SITE: coronary artery ORIGIN: aortic sinus pericarditis myocarditis

Database Correlations

OMIM 611775

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1678
  2. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 93, 1920
  3. 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: 16199713
  4. Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: 19501230
  5. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2022.
  6. 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: 15942913
  7. 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: 20453601
  8. Kole A, Chandakole D IMAGES IN CLINICAL MEDICINE. Strawberry Tongue. N Engl J Med 2015; 373:467. July 30, 2015 PMID: 26222562 http://www.nejm.org/doi/full/10.1056/NEJMicm1411222
  9. Scuccimarri R Kawasaki disease. Pediatr Clin North Am. 2012 Apr;59(2):425-45 PMID: 22560578
  10. 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: 27749951 - Sundel R. Clarifying the Role of Corticosteroids in Kawasaki Disease. JAMA Pediatr. 2016 Oct 17 PMID: 27749946
  11. Cohen E, Sundel R. Kawasaki Disease at 50 Years. JAMA Pediatr. 2016 Sep 26. PMID: 27668809
  12. 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
  13. Alikhan M, Lohr KM (images) Kawasaki Disease: Do You Know the Signs? Medscape. Sept 27, 2017 http://reference.medscape.com/slideshow/kawasaki-disease-6005698
  14. 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: 28356445
  15. NEJM Knowledge+ - Sundel RP Kawasaki disease Rheum Dis Clin North Am. 2015;41(1):63-73 PMID: 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: 24565879