Severe protein losing enteropathy with intractable diarrhea due to systemic AA amyloidosis, successfully treated with corticosteroid and octreotide
Severe protein losing enteropathy with intractable diarrhea due to systemic AA amyloidosis, successfully treated with corticosteroid and octreotide
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DOI：
10.1080/13506120500032725
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发表时间：
2005-03-01
影响因子：
5.5
通讯作者：
Ikeda, SI
中科院分区：
文献类型：
--
作者：
Fushimi, T;Takahashi, Y;Ikeda, SI
本报告涉及两例由AA淀粉样变性引起的严重蛋白质丢失性肠病和难治性腹泻患者，他们成功地使用皮质类固醇和奥曲肽治疗。在这些患者中，胃肠道活检组织显示AA淀粉样蛋白广泛沉积，其中一例由类风湿性关节炎引起，另一例病因不明。两例患者均表现为严重腹泻，对常规治疗无反应，伴低蛋白血症，经tc -99m-二乙烯三胺五乙酸人血清白蛋白（HSA-D）显像证实有蛋白从小肠渗漏至升结肠。在开始使用长效生长抑素类似物奥曲肽和口服强的松龙后不久，他们的总体状况得到改善，水样腹泻量迅速减少，血清白蛋白和IgG水平增加。在Tc-99m-HSA-D闪烁成像上，两名患者的胃肠道蛋白渗漏明显减少。生长抑素类似物和皮质类固醇联合治疗可能对胃肠道淀粉样变性引起的蛋白质丢失性肠病合并难治性腹泻有效。由于在这种严重的临床情况下缺乏特异性的治疗方法，因此应积极考虑将上述治疗方法作为一种治疗选择，不仅适用于AA型淀粉样变性，也适用于其他类型的全身性淀粉样变性。
This report concerns two patients with severe protein losing enteropathy and refractory diarrhea due to AA amyloidosis who were successfully treated with corticosteroid and octreotide. In these patients, biopsied tissues from the gastrointestinal (GI) tract showed extensive deposition of AA amyloid, which was caused by rheumatoid arthritis in one case and was of unidentified etiology in the other. Both patients manifested severe diarrhea unresponsive to conventional treatment with hypoproteinemia, and protein leakage from the small intestine to the ascending colon was confirmed by Tc-99m-diethylene triamine pentaacetic acid human serum albumin (HSA-D) scintigraphy. Soon after starting a long-acting somatostatin analogue, octreotide, with co-administration of oral prednisolone, their general status improved in parallel with a rapid decrease in the volume of watery diarrhea and an increase in serum levels of albumin and IgG. Also on Tc-99m-HSA-D scintigraphy protein leakage from the GI tract was apparently decreased in both patients. Combination therapy with a somatostatin analogue and corticosteroid may be effective for protein losing enteropathy with intractable diarrhea ascribable to GI amyloidosis. Because of the lack of specific therapies in this serious clinical situation, the described therapy should actively be considered as a therapeutic option not only in AA amyloidosis, but also in other types of systemic amyloidosis.