Protein‐losing gastroenteropathy with severe hypoalbuminemia associated with Sj?gren’s syndrome: A case report and review of the literature
Protein‐losing gastroenteropathy with severe hypoalbuminemia associated with Sj?gren’s syndrome: A case report and review of the literature
复制标题
干燥综合征相关蛋白丢失性胃肠病伴严重低蛋白血症：病例报告及文献综述
DOI：
10.1002/jgf2.281
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发表时间：
2019
影响因子：
1.6
通讯作者：
Ishii Tadashi
中科院分区：
文献类型：
--
作者：
Akaishi Tetsuya;Yasaka Ken;Abe Michiaki;Fujii Hiroshi;Watanabe Mika;Shirai Tsuyoshi;Ishizawa Kota;Takayama Shin;Kagaya Yutaka;Harigae Hideo;Ishii Tadashi
患者男，30岁，严重低蛋白血症(血清白蛋白：0.9微克/分升)，双侧小腿严重肿胀，单侧胸腔积液。血清抗SS-A和SS-B抗体水平异常升高，其症状符合干燥综合征的诊断标准。~(99m)Tc-白蛋白核素扫描显示，蛋白质从小肠的大片区域渗漏。免疫组织化学显示十二指肠粘膜血管周围有C1q、C3d和免疫球蛋白G沉积。患者被诊断为与Sjögren综合征相关的蛋白缺失性胃肠病。口服强的松龙和霉酚酸酯治疗2个月内，低蛋白血症和干燥综合征的临床症状完全消失。
A 30‐year‐old man with severe hypoalbuminemia (serum albumin: 0.9 g/dL) was admitted with severe bilateral leg edema and unilateral pleural effusion. Serum anti‐SS‐A and SS‐B antibody levels were abnormally elevated, and his symptoms fulfilled the diagnostic criteria for Sjögren's syndrome. Technetium‐99m albumin scintigraphy revealed protein leakage from a large area of the small intestine. Immunohistochemistry revealed perivascular deposition of C1q, C3d, and immunoglobulin G in the duodenal mucosa. The patient was diagnosed with protein‐losing gastroenteropathy associated with Sjögren's syndrome. Within 2 months of treatment with oral prednisolone and mycophenolate mofetil, the clinical symptoms of hypoalbuminemia and Sjögren's syndrome disappeared completely.