Introduction Kawasaki Disease (KD) is a systematic disease, usually affecting children 6months-5 years old. Optimal therapy is intravenous immune globulin (IVIG) (single dose) and aspirin, in order to prevent development of coronary artery aneurysms. Fever persists in 10–15% of the patients and KD is considered refractory then.
Case Report A 15 months old boy, with unremarkable medical history, was admitted with manifestations and laboratory findings of KD. Initially, IVIG (2g/kg) and aspirin (80mg/kg/24h) were given. Fever insisted and there was a further increase of the PLTs and hemolytic anemia was added. A second dose IVIG (2g/kg) was repeated. As fever remained, pulse IV solu-medrol (30/mg/kg/24h) was given in 3 days. There was a new recur of the fever and of the laboratory findings, thus a third dose of IVIG (2mg/kg) was administered, with complete recession of all manifestations. The extended laboratory investigation revealed positive IgM antibodies for Mycoplasma pneumonia. During the boy’s hospitalization, cardiologic echograms where normal.
Refractory KD cases might respond to 2 or 3 doses of IVIG (2g/kg), with the addition of pulse IV solu-medrol or other immunoregulator agents (e.g. infliximab). Since the disease is very rare, there are few controlled available data about the benefits of treatment.
Mycoplasma pneumoniae is considered as a trigger factor for KD, by producing superantigens, and can also trigger hemolytic anemia.
Though refractory KD is considered as high risk for development of coronary aneurysms, in the case herein, coronary arteries remained intact during all follow up visits.