Hepatic predominant presentation of Kawasaki disease in adolescence case report and review of literature

Abstract

Background Kawasaki Disease (KD) is the most common paediatric vasculitis affecting small to medium arteries. Although the average age of diagnosis is 3.4 years with a well-defined clinical presentation, older patients with KD including adolescent and adult patients demonstrate a less classical presentation with prominent findings including hepatitis, cervical lymphadenopathy, and arthralgia. We describe a case of an adolescent presentation of Kawasaki Disease presenting with a predominantly cholestatic hepatic picture. Case presentation We describe a case of KD in a 16-year-old Caucasian female with predominately hepatic disease that showed resistance to intravenous immunoglobulin (IVIG). The formal diagnosis of KD was made on her 8th day of symptoms. She displayed classical symptoms commencing with fever, followed by peripheral desquamation, strawberry tongue, cervical lymphadenopathy. She became clinically jaundiced with evidence of hepatic artery narrowing on ultrasound that resolved with treatment. Her disease was biphasic and required further IVIG for non-hepatic symptoms. She did not develop coronary aneurysms. Conclusion Significant hepatic dysfunction with clinical jaundice is rare in KD without associated gall bladder hydrops and tends to occur in older patients. We describe such a case and review the five described cases in the literature. Diagnostic delay is more common in adolescent patients and given that the prognosis of KD is closely correlated to diagnostic timing and provision of care, it is important to consider Kawasaki Disease in older demographics especially with undiagnosed hepatic disease.

Jaundice-predominant manifestation of Kawasaki disease in children

“…Furthermore, Vergine et al ( 38 ) and Momenah et al reported that older children above the age of 9 years accounted for 7.5% of their KD patients, and that they had a higher risk of coronary artery abnormalities ( 39 ), especially those who presented with febrile cholestasis. In addition, Pratap et al found that hepatic-predominant manifestations were more common in older children who were more refractory to treatment ( 40 ). Therefore, some experts have concluded that a high level of suspicion should be maintained in children who present with febrile jaundice and atypical KD, especially among older children and adolescents.…”

Dilemma in diagnosing incomplete Kawasaki disease in a resource limited setting

“…There have been various studies and case reports in the past describing the varied manifestations of IKD. Pratap et al 10 described KD in an adult with predominant hepatic involvement which is rare. Hu C et al 11 reported KD in a 4 years old boy with predominant gastrointestinal symptoms with signs of gastrointestinal haemorrhage on Endoscopy.…”