Pasoto Sandra Gofinet, Adriano de Oliveira Martins Victor, Bonfa Eloisa
Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Sao Paulo, Brazil,
Laboratory Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Sao Paulo, Brazil,
Open Access Rheumatol. 2019 Jan 29;11:33-45. doi: 10.2147/OARRR.S167783. eCollection 2019.
Systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS) may coexist, and they are chronic complex disorders, with an autoimmune background, multifactorial etiology, multiple circulating autoantibodies, and variable prognosis. The prominent feature of SS is the impairment of the lacrimal and salivary glands leading to sicca symptoms. This disease may be classified as primary Sjögren's syndrome (pSS), or secondary Sjögren's syndrome (sSS) since it is often associated to other autoimmune disorders, principally SLE, rheumatoid arthritis, and systemic sclerosis. Systematic reviews and meta-analyses show an sSS prevalence in SLE patients of about 14%-17.8%. Herein, we updated important aspects of the clinical association between SLE and sSS through a narrative review of the PubMed database in the last 5 years (from July 2013 to October 2018) with the terms "Sjogren syndrome and systemic lupus erythematosus". The following aspects are addressed: the classification criteria for sSS; differences and similarities between SLE and pSS regarding demographic, clinical, and serological characteristics (including new autoantibodies), as well as comorbidities; the etiopathogenic links between SLE and pSS (including genetic and environmental factors, B-cell activation, and autoantibodies); the predictive factors for sSS onset in SLE patients; the ocular and oral involvements due to sSS in SLE; and the main distinctive demographic, clinical, and serological features of SLE with and without associated SS.
系统性红斑狼疮（SLE）和干燥综合征（SS）可能并存，它们是慢性复杂疾病，具有自身免疫背景、多因素病因、多种循环自身抗体且预后各异。SS的突出特征是泪腺和唾液腺受损导致干燥症状。该疾病可分为原发性干燥综合征（pSS）或继发性干燥综合征（sSS），因为它常与其他自身免疫性疾病相关，主要是SLE、类风湿关节炎和系统性硬化症。系统评价和荟萃分析显示SLE患者中sSS的患病率约为14% - 17.8%。在此，我们通过对过去5年（2013年7月至2018年10月）PubMed数据库中以“Sjogren综合征和系统性红斑狼疮”为关键词的文献进行叙述性综述，更新了SLE与sSS临床关联的重要方面。讨论了以下方面：sSS的分类标准；SLE与pSS在人口统计学、临床和血清学特征（包括新的自身抗体）以及合并症方面的异同；SLE与pSS之间的病因学联系（包括遗传和环境因素、B细胞活化和自身抗体）；SLE患者发生sSS的预测因素；SLE中sSS所致的眼部和口腔受累情况；以及伴有和不伴有相关SS的SLE的主要独特人口统计学、临床和血清学特征。
