Kawasaki disease refractory to standard treatments that responds to a combination of pulsed methylprednisolone and plasma exchange: Cytokine profiling and literature review.

Abstract

UNLABELLED:

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.

CONCLUSION:

PE concurrent with additional anti-inflammatory treatment such as IVMP might be a very promising treatment option for PE refractory patients.

• Authors: Motohiro Matsui, Yoshiaki Okuma, Junko Yamanaka, Hideko Uryu, Noriko Sato, Hiroyuki Shichino, Takeji Matsushita
• Journal: Cytokine (Cytokine) Vol. 74 Issue 2 Pg. 339-42 (Aug 2015) ISSN: 1096-0023 [Electronic] England
• PMID: 25801094 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2015 Elsevier Ltd. All rights reserved.

Chemical References

• IL6 protein, human
• Interleukin-6
• Receptors, Tumor Necrosis Factor, Type I
• Receptors, Tumor Necrosis Factor, Type II
• Methylprednisolone

Topics

• Child, Preschool
• Female
• Humans
• Interleukin-6 (blood)
• Methylprednisolone (administration & dosage)
• Mucocutaneous Lymph Node Syndrome (blood, therapy)
• Plasma Exchange
• Receptors, Tumor Necrosis Factor, Type I (blood)
• Receptors, Tumor Necrosis Factor, Type II (blood)