Primary Sjogren's Syndrome

被引:578
|
作者
Mariette, Xavier [1 ]
Criswell, Lindsey A. [2 ,3 ]
机构
[1] Univ Paris Sud, Dept Rheumatol, Ctr Immunol Viral Infect & Autoimmune Dis, Hop Univ Paris Sud,INSERM,AP HP,Unite 1184, Le Kremlin Bicetre, France
[2] Univ Calif San Francisco, Rosalind Russell Ephraim P Engleman Rheumatol Res, Dept Med, San Francisco, CA 94143 USA
[3] Univ Calif San Francisco, Rosalind Russell Ephraim P Engleman Rheumatol Res, Dept Orofacial Sci, San Francisco, CA 94143 USA
来源
NEW ENGLAND JOURNAL OF MEDICINE | 2018年 / 378卷 / 10期
关键词
CONTROLLED-TRIAL; DOUBLE-BLIND; CLINICAL-TRIAL; RITUXIMAB; PATHOGENESIS; AUTOIMMUNE; SIGNATURES; CONSENSUS; EFFICACY; DISEASES;
D O I
10.1056/NEJMcp1702514
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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?
引用
收藏
页码:931 / 939
页数:9
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