Matsui Motohiro, Okuma Yoshiaki, Yamanaka Junko, Uryu Hideko, Sato Noriko, Shichino Hiroyuki, Matsushita Takeji
Division of Pediatrics, National Center for Global health and Medicine, 1-21-1 Toyama, Shinjyuku-ku, Tokyo, Japan.
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.
Cytokine. 2015 Aug;74(2):339-42. doi: 10.1016/j.cyto.2015.02.014. Epub 2015 Mar 19.
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.
PE concurrent with additional anti-inflammatory treatment such as IVMP might be a very promising treatment option for PE refractory patients.
我们报告一例川崎病（KD）患者，该患者对血浆置换（PE）治疗无效，但最终对静脉注射甲泼尼龙冲击治疗（IVMP）与PE联合治疗产生了反应。为了确定这种联合治疗有效性的直接和间接证据，我们通过使用医学数据库检索文献和进行细胞因子分析，对难治性KD患者的数据进行了分析。在文献检索中，我们使用了PubMed™和Ichushi™数据库。所用检索词包括“川崎病”和“血浆置换”，以提取描述接受PE治疗的KD病例的文章。对于细胞因子分析，我们在PE治疗前、后以及PE与IVMP联合治疗前、后测量了白细胞介素（IL）-6、可溶性肿瘤坏死因子-α受体（sTNF-αR）1型和2型。我们的检索发现2例接受PE治疗的KD患者，其中PE治疗对188例患者有效（93.5%），但对13例患者无效（6.5%）。所有13例患者均通过额外治疗成功治愈。在这13例患者中，仅6例（2.5%）在PE治疗期间复发。在我们的病例中，细胞因子分析显示PE治疗使IL-6降低，而sTNF-αR 1型和2型仍处于高水平。PE与IVMP联合治疗使IL-6以及sTNFα-R 1型和2型水平降低。
PE与IVMP等额外抗炎治疗联合使用可能是PE难治性患者非常有前景的治疗选择。
