Abstract
Kawasaki disease is an acute multisystemic vasculitis occurring predominantly in infants and young children and rarely in adolescents and adults. At elderly age, Kawasaki disease may remain unrecognized with a subsequent delay in appropriate therapy and an increased risk of coronary artery aneurysms. We report a case of intravenous immunoglobulin- and aspirin-resistant Kawasaki disease and severe cardiovascular damage in an adolescent boy. The article discusses major issues associated with the management of refractory Kawasaki disease.
Highlights
Kawasaki disease (KD) is an acute necrotizing vasculitis of medium- and small-sized vessels with a life-threatening predisposition to involve coronary arteries, predominantly occurring in children aged from 6 months to 5 years [1, 2]
Though the diagnosis was made on the first day of hospitalization and the treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin was started, the patient failed to defervescence, and additional therapy with steroids and antitumor necrosis factor alpha was administered
There is no specific diagnostic test available for KD; the diagnosis in both adults and children is based on the presence of characteristic findings, i.e., fever lasting for 5 days or more and at least 4 of the 5 following clinical signs: 1) nonexudative bilateral conjunctivitis; 2) diffuse erythema of the oropharyngeal mucosa, fissured lips, or strawberry tongue; 3) cervical adenopathy; 4) polymorphic rash; and 5) erythema or edema of the palms and the soles progressing to a periungual desquamation in the subacute phase
Summary
Kawasaki disease (KD) is an acute necrotizing vasculitis of medium- and small-sized vessels with a life-threatening predisposition to involve coronary arteries, predominantly occurring in children aged from 6 months to 5 years [1, 2]. In view of persisting systemic inflammation and coronary artery damage, infliximab at a dose of 5 mg/ kg (400 mg) was given This treatment was effective, and the patient showed defervescence within 1 day; he had no complaints and laboratory values were within reference ranges. A gated rest test and a myocardial perfusion scan were performed with intravenous 99mTc MIBI (methoxyisobutyl isonitrile) according to the standard protocols (gamma camera equipped with low-energy high-resolution collimators, Siemens E-CAM, Germany) This examination did not reveal the signs of perfusion abnormality but showed inhomogeneous perfusion in the segments of the LV (Fig. 3), nonsynchronous contraction of the LV, insufficient thickening of the basal and middle segments of the septum, and decreased LV EF (56%).
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