进展为系统性红斑狼疮的干燥综合征患者临床和实验室特点

Clinical and Laboratory Characteristics of Sjgren's Syndrome that Progressed to Systemic Lupus Erythematosus

目的探讨以干燥综合征(Sjgren’s syndrome,SS)起病、进展为系统性红斑狼疮(systemic lupus erythematosus,SLE)患者临床和实验室的相关影响因素。方法采取回顾性病例对照研究的方法,选取1997年1月至2012年2月北京协和医院55例以原发性干燥综合征(primary Sjgren’s syndrome,pSS)起病、后进展为SLE的住院患者为SS-SLE组,随机抽取同期该医院不合并其他自身免疫性疾病pSS住院患者55例为pSS组,就两组的临床特点和实验室检查进行单因素和多因素Logistic回归分析。结果 SS-SLE组和PSS组患者在平均发病年龄[(31±23)岁vs.(39±22)岁]、口干症(78.2%vs.96.4%)、关节肿痛(74.5%vs.40.0%)、肺间质病变(27.3%vs.54.5%)、白细胞下降(56.4%vs.29.1%)、蛋白尿增多(27.3%vs.7.3%),以及补体CH50(30.9%vs.1.8%)、C3(54.5%vs.12.7%)和C4(41.8%vs.7.3%)下降等方面的差异均有统计学意义(均P<0.05)。多因素Logistic回归分析提示SS发展为SLE的独立危险因素为年龄、C3、C4。结论 SS-SLE患者发病年龄较早,关节炎、白细胞减少、蛋白尿增多和补体下降的发生率较高,口干症、肺间质病变发生率较低。其中以年龄及补体C3、C4下降为独立危险因素。部分SS可以先于SLE发生,发病年龄较早、存在补体下降的SS患者进展为SLE的风险更高,该类患者出现白细胞减少、蛋白尿、关节肿痛的可能性更高,临床上需警惕具有上述临床特点的SS患者进展为SLE。

Objective To investigate the risk factors for Sj6gren＇ s syndrome （SS） that developed to systemic lupus erythematosus （SLE）. Methods The clinical and laboratory characteristics of 55 cases of SS-SLE （ pSS when onset, then progressed to SLE） and 55 patients with pSS （ not associated with other autoimmune diseases） were analyzed. Results There were significant differences （P 〈 0.05 ） between SS-SLE and pSS patients in： the mean age at onset： （31 _＋23）years old vs （39 _＋22） years old; the clinical manifestations： xerostomia （ 78.2% vs 96.4% ）, arthritis （ 74.5% vs 40. 0% ）, interstitial lung disease （ 27.3.0% vs 54.5% ）. laboratory test results： leukopenia （56.4% vs 29. 1% ）, increased proteinuria （27.3% vs 7.3%）, decline of complement [including CHS0 （30.9% vs 1.8%）, C3（54.5% vs 12.7%）, C4 （41.8% vs 7.3% ） ] . Logistic regression analysis showed that younger age at disease onset and decline of C3 and C4 were independent risk factors. Conclusions The SS patients who progressed to SLE have earlier age of disease onset, higher incidence of arthritis, leucopenia, proteinuria and complement decline, lower incidence of dry mouth and interstitial lung disease. We should be vigilant to those patients of SS who have the clinical and laboratory characteristics identified by this study, may progress to SLE.