2020 Volume 90 Issue 6 Pages 126-131
We report a case of Kawasaki disease (KD) complicated by coronary artery lesions (CALs) with various symptoms other than major items, such as arthralgia and nail lesions.
Case: A 23-month-old girl developed fever. Five out of 6 major items of KD were confirmed on the day of illness 3, and the patient was hospitalized with a diagnosis of KD. She did not respond to the first dose of high-dose immunoglobulin therapy (IVIg). Knee and hip joint pain appeared and orange-brown chromonychia, a characteristic nail finding of KD, was recognized on day 9. After additional IVIg and cyclosporine A (CsA), she developed recurrent fever on day 16, and moderate CALs were observed. The joint pain worsened with fever and were alleviated as the fever improved. Finally, she required 5 doses of IVIg, CsA for 39 days, moderate doses of aspirin for 48 days, and antihypertensive medication. The left anterior descending artery measured a maximum of 4.1 mm (Z score+6.6). She had desquamation of her fingers on day 14, and the joint pain almost disappeared on day 50. She was discharged on day 54. The CALs improved well after 3 months.
Discussion: Systemic juvenile idiopathic arthritis was also considered as a differential diagnosis by the joint pain, but she was finally diagnosed as KD because of BCG inoculation redness, nail findings, membranous desquamation of hand and foot, laboratory test values, and final resolve of fever. In this case, the responsiveness to IVIg and CsA was insufficient. There are several treatment options for IVIg-resistant KD, and it is necessary to establish treatment selection criteria.
