| H01718 | |
| H number | H01718 |
| Name | Kawasaki disease; Mucocutaneous lymph node syndrome |
| Description | Kawasaki disease (KD) is an acute systemic vasculitis of childhood that does not have a known cause or aetiology. KD is a self-limited illness that is not associated with the production of autoantibodies or the deposition of immune complexes, and it rarely recurs. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, accompanied by four out of five findings: bilateral conjunctival injection, oral changes such as cracked and erythematous lips and strawberry tongue, cervical lymphadenopathy, extremity changes such as erythema or palm and sole desquamation, and polymorphous rash. Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of these findings. The standard treatment of acute KD is intravenous immunoglobulin (IVIG) infusion and aspirin. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis. If there is no response to treatment, patients are given a second dose of IVIG with or without corticosteroids or other adjunctive treatment. |
| Category | Immune system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | - |
| Other DBs | ICD-11: 4A44.5 ICD-10: M30.3 MeSH: D009080 |
| Reference | PMID:27342882 AUTHORS Hara T, Nakashima Y, Sakai Y, Nishio H, Motomura Y, Yamasaki S TITLE Kawasaki disease: a matter of innate immunity. JOURNAL Clin Exp Immunol 186:134-143 (2016) DOI:10.1111/cei.12832 PMID:27506874 AUTHORS Galeotti C, Kaveri SV, Cimaz R, Kone-Paut I, Bayry J TITLE Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease. JOURNAL Drug Discov Today 21:1850-1857 (2016) DOI:10.1016/j.drudis.2016.08.004 PMID:25822554 AUTHORS Saguil A, Fargo M, Grogan S TITLE Diagnosis and management of kawasaki disease. JOURNAL Am Fam Physician 91:365-71 (2015) PMID:24701096 AUTHORS Rodriguez-Gonzalez M, Matamala-Morillo MA, Segado-Arenas A TITLE Infliximab as rescue therapy in refractory Kawasaki disease. JOURNAL Ann Pediatr Cardiol 7:74-5 (2014) DOI:10.4103/0974-2069.126578 |