A THREE-YEAR-OLD CHILD WITH LATE-ONSET HYPERTENSION AND RENAL ARTERY INVOLVEMENT: IS THIS DISEASE KAWASAKI DISEASE OR ANOTHER TYPE OF VASCULITIS?

Background: Kawasaki disease (KD) is an acute multisystemic vasculitis with particular importance being the most common cause of acquired heart disease of childhood. Approximately 20% of untreated children will develop coronary artery abnormalities. However, coexistence of Kawasaki disease with hypertension and renal artery involvement has not been well investigated. In this case report, we present a three year old hypertensive girl with stenosis of intracranial and intrarenal arcuate arteries and coexisting KD.In this case report, we present a three year old hypertensive girl with stenosis of intracranial and intrarenal arcuate arteries and coexisting KD.Case report: A three year old girl with high fever unresponsive to antipyretics and antibiotics presented with a maculo-papular rash on the trunk and upper extremities. Ten days later, red fissured lips, a strawberry tongue and bulbar conjunctival injection was recognized. Intravenous immunoglobulin (2gr/kg) and aspirin were administered following the diagnosis of KD. The blood pressure was within the normal limits. High fever persisted despite two doses of IVIG therapy and pulse corticosteroid therapy (30 mg/kg/per dose) was given. Echocardiography was normal. Following pulse corticosteroid therapy, fever disappeared; but, the child presented with high fever in two days. Proximal interphalangial and metacarpophalangial joints were swollen with apparent rubor and bilateral loss of function. Because of long lasting fever, positivity of acute phase reactants, thrombocytosis and joint findings, the diagnosis was revised to be systemic JIA and corticosteroids and methotrexate (10 mg/m) were administered. Under this treatment regimen, high fever and laboratory findings persisted; but, blood pressure was within normal limits. Two months later the patient presented with vomiting, headache and convulsion. This time a high blood pressure was measured (135/95 mmHg). The angiography of the patient showed stenosis of celiac truncus, superior mesenteric artery and right renal artery with impaired perfusion. Additionally, segmental stenotic areas and contour irregularities were observed in intracranial and bilateral middle to small intrarenal arteries. Cyclophosphamide, azothiopurine and enalapril were added for the treatment. The symptoms and signs disappeared and the blood pressure was back to normal.Conclusion: In the absence of coronary artery involvement, KD still holds the potential to cause aneurysmatic or stenotic changes in intracranial and/or middle to small renal arteries due to long-standing inflammation. This clinical picture could be another vasculitic syndrome other than KD.Citation: Ann Rheum Dis, volume 65, supplement II, year 2006, page 440Session: Paediatric rheumatology