Tokura Masami, Niwano Tomoko, Nagasaka Kenji
Department of Rheumatology, Ome Municipal General Hospital, Ome, Japan.
Case Rep Rheumatol. 2019 Mar 4;2019:4865024. doi: 10.1155/2019/4865024. eCollection 2019.
A 70-year-old woman with Sjögren's syndrome (SS) complained of generalized edema. Computed tomography showed thoracoabdominal fluid, suggesting serositis with SS. 35 mg/day of prednisolone as a monotherapy was ineffective. Moreover, hemoconcentration with hypoalbuminemia without inflammatory signs lead us to consider the systemic capillary leak syndrome (SCLS). Additional treatment with intravenous immunoglobulin (IVIG) and prednisolone dramatically decreased the thoracoabdominal fluid. However, when reducing the prednisolone dose, the thoracoabdominal fluid reincreased. Retreatment with IVIG without increasing the prednisolone dose was ineffective. However, additional prednisolone of 35 mg/day was effective, suggesting SCLS with SS might require combination therapy with IVIG and glucocorticoid.
一名患有干燥综合征（SS）的70岁女性主诉全身水肿。计算机断层扫描显示胸腹有积液，提示患有SS的浆膜炎。单独使用泼尼松龙35毫克/天治疗无效。此外，存在血液浓缩伴低白蛋白血症且无炎症迹象，这使我们考虑系统性毛细血管渗漏综合征（SCLS）。静脉注射免疫球蛋白（IVIG）和泼尼松龙联合治疗使胸腹积液显著减少。然而，当降低泼尼松龙剂量时，胸腹积液再次增加。在不增加泼尼松龙剂量的情况下再次使用IVIG治疗无效。然而，额外给予35毫克/天的泼尼松龙有效，这表明患有SS的SCLS可能需要IVIG和糖皮质激素联合治疗。
