Abstract 142: Plasma Exchange Therapy in a Patient with Refractory Kawasaki Disease Initially Treated with Intravenous Immunoglobulin and Prednisolone
Prednisolone combined with intravenous immunoglobulin (IVIG) is the standard option for the initial treatment of Kawasaki disease (KD) in Japan, as described in the 2012 Japanese guidelines for KD. However, there is still debate regarding treatment in patients with refractory KD initially treated with IVIG plus prednisolone, especially in those with comorbid coronary artery aneurysm (CAA). Here, we present the case of a 3 year-old-girl initially treated with IVIG plus prednisolone, whose clinical and laboratory parameters improved after plasma exchange therapy (PE). She was admitted to a local hospital on day 4 of illness and diagnosed as having severe KD (Kobayashi score, 7). Therefore, prednisolone plus IVIG was administered as initial treatment. The initial course was ineffective. She was treated with methylprednisolone pulse therapy, and a second and third round of additional IVIG, but low-grade fever persisted. After these treatments the patient’s general condition had improved and major KD symptoms improved by day 13 of illness, despite a high serum C-reactive protein level (19.9 mg/dl) and hypoalbuminemia (1.8 g/dl). Expanding medium-sized aneurysm was detected on the same day, so she was immediately referred to our hospital for further treatment. The CAA continued to expand, so PE was performed for 3 consecutive days and prednisolone was gradually reduced from day 15 of illness. After that, she became afebrile and CAA progression stopped. Laboratory findings such as serum C-reactive protein and albumin levels were dramatically improved after PE. This case suggests that PE might be effective for refractory KD initially treated with IVIG plus glucocorticoids and may stop the CAA development. Doctors must remain aware that patients treated with glucocorticoids may have masked clinical conditions, and take care not to underestimate the signs of refractory KD.
Author Disclosures: K. Okunushi: None. R. Ebata: None. N. Hattori: None. H. Kobayashi: None. N. Saito: None. S. Oda: None. N. Shimojo: None.
- © 2015 by American Heart Association, Inc.