Suppr超能文献

干燥综合征-红斑狼疮综合征的重叠

[Overlap of Sjögren-lupus erythematosus syndrome].

作者信息

Trüeb R M, Borelli S, Schmid-Grendelmeier P, Panizzon R G, Burg G

机构信息

Dermatologische Klinik, Universitätsspital, Zürich.

出版信息

Hautarzt. 1995 Jul;46(7):467-71. doi: 10.1007/s001050050283.

Abstract

Patients with anti-SSA(Ro)-positive Sjögren's syndrome (SS)/lupus erythematosus (LE) overlap are a immunogenetically, serologically and clinically homogeneous group; what they have in common is an increased frequency of the HLA-DR3 phenotype, demonstrating SSA(Ro) antibody activity and the typical annular, polycyclic, erythematous lesions of subacute cutaneous LE. The sicca symptoms of SS may develop long after the cutaneous lesions of LE have been present, or vice versa, as well as vasculitic, purpuric, and Sweet's syndrome-like lesions. The elderly, predominantly female patients are at enhanced risk for pulmonary and neurological disease, but glomerulonephritis occurs infrequently. We present an 81-year-old, SSA(Ro) antibody-positive and HLA-DR3-positive woman with SS/LE overlap syndrome, who had cutaneous LE for 30 years, developing sicca symptoms, vasculitic and Sweet's syndrome-like skin lesions only years after the LE symptoms. Although there is still some discussion as to whether phenotypical SS/LE overlap represents a distinct disease or is secondary SS in SLE or systemic SS with cutaneous involvement, the SS/LE overlap syndrome seems well enough delineated to be considered as a disease entity with separate implications for therapy and prognosis. Its position in the spectrum of anti-SSA(Ro) antibody- and HLA-DR3-positive diseases and its relationship to SS and to anti-SSA(Ro) antibody-positive LE are discussed.

摘要

抗SSA(Ro)抗体阳性的干燥综合征(SS)/红斑狼疮(LE)重叠综合征患者在免疫遗传学、血清学和临床上是一个同质群体;他们的共同特点是HLA - DR3表型频率增加,表现出SSA(Ro)抗体活性以及亚急性皮肤型LE典型的环形、多环形红斑性皮损。SS的干燥症状可能在LE皮肤损害出现很久之后才出现,反之亦然,还可能出现血管炎、紫癜和类Sweet综合征样皮损。老年患者(以女性为主)发生肺部和神经系统疾病的风险增加,但肾小球肾炎很少见。我们报告一名81岁抗SSA(Ro)抗体阳性且HLA - DR3阳性的女性,患有SS/LE重叠综合征,有30年的皮肤型LE病史,在LE症状出现数年之后才出现干燥症状、血管炎和类Sweet综合征样皮肤损害。尽管对于表型SS/LE重叠是代表一种独特疾病还是SLE中的继发性SS或伴有皮肤受累的系统性SS仍存在一些讨论,但SS/LE重叠综合征似乎已得到充分界定,可被视为一种具有独特治疗和预后意义的疾病实体。本文讨论了它在抗SSA(Ro)抗体和HLA - DR3阳性疾病谱中的位置及其与SS以及抗SSA(Ro)抗体阳性LE的关系。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验