干燥综合征的临床特点、预后及死因分析
发表者： 金银姬
日期：2015-02-27
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摘要：本回顾性单中心研究对547名诊为原发性干燥综合征的患者临床及实验室指标和疾病转归进行了分析。随访（11.4 ± 6.2）年。我们评估了临床及实验室指标，并分析其对诊断时间、预后及死亡率的影响。对干燥综合症患者性别、腺体与腺外表现、相关疾病及异常血清免疫学指标进行分析。结果显示，最常见的腺外表现为多关节炎、雷诺现象及血管炎。最常见的相关疾病为甲状腺炎。随访期间，有51例患者死亡，中位生存时间33.71年。结果还显示,冷球蛋白血症可降低生存率。另外，血管炎及淋巴组织增生性疾病可使死亡风险增高。有腺外表现患者的死亡率为仅有腺体受累患者的2-3倍。 针对死亡风险高的原发性干燥综合征患者，强调密切监测和诊断方针。
附原文：Abstract The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4 ± 6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk.
引自：Horvath IF，Szanto A，Papp G，et al. Clinical Course, Prognosis, and Cause of Death in Primary Sjögren's Syndrome. J Immunol Res. 2014;2014:647507. doi: 10.1155/2014/647507. Epub 2014 May 20. （注：干燥综合症病人或亲属可加QQ群交流，群号： 118194945 ，本网站站长私人微信号： ssgzz88 ）
附原文：Abstract The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4 ± 6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk.
引自：Horvath IF，Szanto A，Papp G，et al. Clinical Course, Prognosis, and Cause of Death in Primary Sjögren's Syndrome. J Immunol Res. 2014;2014:647507. doi: 10.1155/2014/647507. Epub 2014 May 20. （注：干燥综合症病人或亲属可加QQ群交流，群号： 118194945 ，本网站站长私人微信号： ssgzz88 ）