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干燥综合征

Sjogern's syndrome.

作者信息

Connolly M K

机构信息

Department of Dermatology, University of California, San Francisco 94143-0517, USA.

出版信息

Semin Cutan Med Surg. 2001 Mar;20(1):46-52. doi: 10.1053/sder.2001.23096.

DOI:10.1053/sder.2001.23096
PMID:11308136
Abstract

Sjogren's syndrome (SS) is a systemic autoimmune exocrinopathy that affects the salivary and lacrimal glands. It typically presents as the "sicca complex" of dry eyes (xerophthalmia) and dry mouth (xerostomia) along with other symptoms such as arthritis. SS is classified as either primary or secondary. In the primary form, dry eyes and dry mouth occur alone. In the secondary form, the dry eyes and dry mouth occur in the context of another rheumatic disease, most commonly rheumatoid arthritis. There is an increasing list of systemic manifestations affecting the lung, kidney, and nervous system in patients with SS. The skin is affected in half of SS patients. Despite this high frequency of cutaneous involvement, patients with SS are not commonly seen in dermatology practices. SS is underrecognized and underdiagnosed because the cutaneous manifestations are nonspecific (eg, xerosis, pruritus) and less severe than the oral, ocular, or musculoskeletal symptoms. Nonetheless, because of its high prevalence, risk of cutaneous vasculitis, and the increased risk of a lymphoproliferative disorder, it is important for dermatologists to be familiar with SS.

摘要

干燥综合征(SS)是一种影响唾液腺和泪腺的全身性自身免疫性外分泌腺病。其典型表现为眼干(干眼症)和口干(口干燥症)的“干燥综合征”,以及关节炎等其他症状。SS分为原发性或继发性。原发性形式中,单独出现眼干和口干。继发性形式中,眼干和口干出现在另一种风湿性疾病的背景下,最常见的是类风湿关节炎。SS患者出现影响肺部、肾脏和神经系统的全身性表现的情况越来越多。一半的SS患者皮肤会受到影响。尽管皮肤受累的频率很高,但SS患者在皮肤科门诊并不常见。SS未得到充分认识和诊断,因为皮肤表现是非特异性的(如皮肤干燥、瘙痒),且不如口腔、眼部或肌肉骨骼症状严重。然而,由于其高患病率、皮肤血管炎风险以及淋巴增殖性疾病风险增加,皮肤科医生熟悉SS很重要。

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