Primary Sjogren's Syndrome
Primary Sjogren's Syndrome
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DOI：
10.1056/nejmcp1702514
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发表时间：
2018-03-08
影响因子：
158.5
通讯作者：
Criswell, Lindsey A.
中科院分区：
文献类型：
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作者：
Mariette, Xavier;Criswell, Lindsey A.
一位52岁女性，有两年口干史。她吞咽干食物有困难，而且整晚都要喝水。她还报告说，她的手和手腕出现阵发性疲劳和疼痛，特别是在早上。在出现前十年，眼睛不适和干燥导致她停止使用隐形眼镜。在过去的两年里，她有几次腮腺肿胀的发作。体格检查显示口干，腿部可触到紫疹，三个关节肿胀，双侧腮腺肿胀。实验室研究显示，淋巴细胞减少(每立方毫米850个细胞)没有其他血细胞计数异常，血清肌酐水平为每分升1.6毫克(140微克/升；1年前为每升0.7毫克[60克/升])，多克隆伽马病，类风湿因子阳性，存在抗核抗体(包括针对干燥综合征相关抗原A的抗体[抗SSA抗体])，以及不存在冷球蛋白血症的低C4水平。这位患者的病例应该如何处理？
A 52-year-old woman presents with a 2-year history of an extremely dry mouth. She has difficulty swallowing dry food and has to drink water throughout the night. She also reports having episodes of fatigue and pain in her hands and wrists, particularly in the morning. Ten years before presentation, ocular discomfort and dryness caused her to discontinue the use of contact lenses. She has had several episodes of swelling of the parotid glands during the past 2 years. The physical examination reveals dry mouth, palpable purpura on the legs, three swollen joints, and bilateral swelling of the parotid glands. Laboratory studies reveal lymphocytopenia (850 cells per cubic millimeter) without other abnormalities in the blood count, a serum creatinine level of 1.6 mg per deciliter (140 mu mol per liter; as compared with 0.7 mg per deciliter [60 mol per liter] 1 year earlier), polyclonal gammopathy, positive rheumatoid factor, the presence of antinuclear antibodies (including antibodies against Sjogren's syndrome-related antigen A [anti-SSA antibodies]), and a low C4 level without cryoglobulinemia. How should this patient's case be managed?