Kobayashi Yuya, Shimojima Yasuhiro, Kondo Yasufumi, Takamatsu Ryota, Miyazaki Daigo, Kishida Dai, Sekijima Yoshiki, Ikeda Shu-Ichi
Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Japan.
Intern Med. 2017;56(15):2057-2062. doi: 10.2169/internalmedicine.56.8391. Epub 2017 Aug 1.
We herein report the case of a 44-year-old woman who developed protein-losing gastroenteropathy (PLGE) with hypoalbuminemia as the first manifestation of mixed connective tissue disease (MCTD). Albumin leakage from the stomach and intestinal tract was demonstrated by Tc-labeled human serum albumin scintigraphy. The patient's response to prednisolone therapy was insufficient; therefore, additional cyclosporin A (CsA) treatment was administered, and clinical remission was achieved. We concluded that although PLGE is a rare complication of MCTD, it may manifest as an initial clinical episode of MCTD. Furthermore, CsA can be a useful treatment option for refractory PLGE related to MCTD.
我们在此报告一例44岁女性,其以蛋白丢失性胃肠病(PLGE)伴低白蛋白血症作为混合性结缔组织病(MCTD)的首发表现。锝标记的人血清白蛋白闪烁扫描显示胃和肠道有白蛋白渗漏。患者对泼尼松龙治疗反应不足;因此,加用环孢素A(CsA)治疗,实现了临床缓解。我们得出结论,虽然PLGE是MCTD的罕见并发症,但它可能表现为MCTD的初始临床发作。此外,CsA可能是治疗与MCTD相关的难治性PLGE的有用选择。