title: Kawasaki disease refractory to standard treatments that responds to a combination of pulsed methylprednisolone and plasma exchange: Cytokine profiling and literature review.
abstract: We present a case of Kawasaki Disease (KD) that was refractory to plasma exchange (PE), but which finally responded to concurrent intravenous methylprednisolone pulse (IVMP) and PE treatment. To determine direct and indirect evidence for the efficacy of this combination therapy, we analyzed data of patients with refractory KD by review of the literature using medical databases and cytokine profiling. For literature searches, we used the Pubmed™ and Ichushi™ databases. Search terms used included "Kawasaki disease" and "plasma exchange" to extract articles that described KD cases treated with PE. For cytokine profiling, we measured interleukin (IL)-6, soluble tumor necrosis factor-α receptor (sTNF-αR) type 1 and type 2 before and after PE and PE with IVMP. Our search revealed 201 KD patients treated with PE, of which PE treatment was effective in 188 patients (93.5%), but not in 13 cases (6.5%). All 13 cases were treated successfully with additional treatment. Of the 13 cases, only six (2.5%) had recurrence during the PE treatment period. In our case, cytokine profiling showed PE treatment decreased IL6, while sTNF-αR type1 and type2 remained at high levels. PE and IVMP decreased IL-6 and sTNFα-R type 1 and type 2 levels.
  
  
  CONCLUSIONS
  PE concurrent with additional anti-inflammatory treatment such as IVMP might be a very promising treatment option for PE refractory patients.
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan. Electronic address: motohiro612@yahoo.co.jp.
authors: Motohiro
authors: M
authors: Matsui
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan; Advanced and Specialized Medicine, Juntendo University Graduate School of Medicine, Japan.
authors: Yoshiaki
authors: Y
authors: Okuma
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan.
authors: Junko
authors: J
authors: Yamanaka
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan; Advanced and Specialized Medicine, Juntendo University Graduate School of Medicine, Japan.
authors: Hideko
authors: H
authors: Uryu
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan.
authors: Noriko
authors: N
authors: Sato
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan.
authors: Hiroyuki
authors: H
authors: Shichino
authors: Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan.
authors: Takeji
authors: T
authors: Matsushita
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D015850:Interleukin-6 / Q000097:blood*; D008775:Methylprednisolone / Q000008:administration & dosage*; D009080:Mucocutaneous Lymph Node Syndrome / Q000097:blood / Q000628:therapy; D010951:Plasma Exchange; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000097:blood*; D047889:Receptors, Tumor Necrosis Factor, Type II / Q000097:blood*
chemical_list: C508600:IL6 protein, human; D015850:Interleukin-6; D047888:Receptors, Tumor Necrosis Factor, Type I; D047889:Receptors, Tumor Necrosis Factor, Type II; D008775:Methylprednisolone
keywords: Cytokine; Kawasaki disease; Methylprednisolone; Plasma exchange; Refractory