Abstract 126: A Case Of Slowly Progressing Giant Coronary Aneurysm In A Patient With Intractable Kawasaki Disease

• Published in: Circulation (New York, N.Y.) N.Y.), 2015-04, Vol.131 (suppl_2)
• Main Authors: Han, Ji Whan, Seo, Yu Mi, Lee, Kyu Jin, Kim, Joo Hyun, Lee, Jae Young, Oh, Jin Hee, Lee, Soon Ju
• Format: Article
• Language: English
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/circ.131.suppl_2.126

Abstract only Background: Some patients with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) do not respond to intravenous methylprednisolone (IVMP). Infliximab (IFX; anti-TNF-α antibody) is an alternative medication for intractable KD. Its effectiveness in preventing coronary aneurysms from progressing is being reported. We herein report a case of intractable KD that slowly progressed to giant aneurysm despite a second IFX infusion. Methods: A 2-year-old boy visited our hospital with a 2-day history of fever. On day 3 after fever onset, a swollen cervical lymph node, redness of the BCG (bacilli Calmette Guérin) inoculation site, and indurated erythema of the palms with soles were observed. On day 5, conjunctival injection, strawberry tongue, red lips and truncal rash were present. The patient was diagnosed with KD and treated with IVIG and acetylsalicylic acid (ASA). Results: The fever persisted for 24 h after IVIG treatment. A second dose of IVIG was infused. However, the fever continued. Then IVMP was infused. On day 9, the fever resolved. On day 10, two-dimensional echocardiography (2DE) showed mild dilations (Φ 3.0 mm) of both coronary arteries (CAs). On day 18, the fever recurred. Follow-up 2DE showed dilated CAs (left main coronary artery [LM], Φ 4.0 mm; right coronary artery [RCA], Φ 4.0 mm). Therefore, IFX was intravenously infused. On day 19, the fever had resolved and the patient went home. On day 22, the fever recurred. Thus, a second infusion of IFX was done. On day 24, the fever resolved. Follow-up 2DE showed that the diameters of the CAs were 4.0 mm (LM), 7.0 mm (left anterior descending coronary artery [LAD]), 3.0 mm (RCA). On day 28, a mild fever was still present. On day 47, follow-up 2DE indicated that the diameters of the CAs were 3.0 mm (LM), 8.0 mm (LAD), 2.5 mm (RCA). On day 159, follow-up 2DE showed slightly increased regression of CAs. The patient has been undergoing regular 2DE and taking ASA since he left the hospital. Conclusions: This was a very rare case of KD that was resistant to standard treatment comprising high-dose steroids and TNF-α blockade. We have reported this case to inform clinicians who may encounter such patients with KD in the future. A high index of suspicion and keen observations are strongly recommended for cases of intractable KD.