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Clinical and prognostic characteristics of 573 cases of primary Sj(o)gren's syndrome

LIN Dong-fang YAN Shu-min ZHAO Yan ZHANG Wen LI Meng-tao ZENG Xiao-feng ZHANG Feng-chun DONG Yi

中华医学杂志(英文版)2010,Vol.123Issue(22):3252-3257,6.
中华医学杂志(英文版)2010,Vol.123Issue(22):3252-3257,6.DOI:10.3760/cma.j.issn.0366-6999.2010.22.015

Clinical and prognostic characteristics of 573 cases of primary Sj(o)gren's syndrome

Clinical and prognostic characteristics of 573 cases of primary Sj(o)gren's syndrome

LIN Dong-fang 1YAN Shu-min 2ZHAO Yan 3ZHANG Wen 1LI Meng-tao 4ZENG Xiao-feng 4ZHANG Feng-chun 4DONG Yi4

作者信息

  • 1. Department of Rheumatology,Peking Union Medical College Hospital,Peking Union Medical College & Chinese Academy of Medical Sciences,Beijing 100730,China
  • 2. Department of Rheumatology,Third Affiliated Hospital of Sun Yat-sen University,Guangzhou,Guangdong 510630,China
  • 3. Department of Rheumatology,Beijing Jishuitan Hospital,Beijing 100035,China
  • 折叠

摘要

Abstract

Background Primary Sj(o)gren's syndrome (pSS) is one of the autoimmune diseases with high incidence. There were several clinical investigations in Caucasian but seldom in Chinese. The aim of this study was to compare the difference of clinical manifestations, immunological features and prognosis of pSS between Caucasian and Chinese pSS patients.Methods Five hundred and seventy-three patients who fulfilled the 2002 international classification (criteria) for pSS from Peking Union Medical College Hospital between 1985 and 2006 were screened retrospectively and compared with other populations.the onset of the disease was (39.0±13.7) years and in 169 (29.5%) patients the disease onset occurred before the age of 30 years. The average duration from disease onset to pSS diagnosis was 48 months (range, 1-552 months). It had been shortened during the recent five years. (2) Dry mouth (84.5%) and dry eyes (70.0%) were the most common symptoms,significantly lower than foreign patients (P=0.000). Two hundred and seventy-two (47.5%) patients presented with rampant caries, 160 (27.9%) with parotidomegaly. The positivity of xerostomia, xerophthalmia and salivary gland biopsy were 91.9%, 94.8% and 90.7%, respectively. (3) Systemic involvement occurred in 91.4% patients. Compared with studies done outside China, higher prevalence of fever 41.0%, myositis 4.9%, pericardial effusion 14.8%, pulmonary involvement 42.3%, renal involvement 33.5%, thyroid involvement 32.7%, pancrease involvement 5.6% (P <0.01) and lower prevalence of fatigue, lymphadenectasis and Raynaud's phenomenon (P <0.01) were seen. (4) Risk factors of death include pulmonary artery hypertension, liver damage and interstitial lung disease.Conclusions Chinese pSS differs significantly from the non-Chinese cases in terms of the age of onset, systemic involvement, autoantibodies and proportional mortality rate. Lung and liver damage were found to be the highest risk factors of the disease prognosis.

关键词

Sj(o)gren's syndrome/prevalence/prognosis

Key words

Sj(o)gren's syndrome/prevalence/prognosis

分类

医药卫生

引用本文复制引用

LIN Dong-fang,YAN Shu-min,ZHAO Yan,ZHANG Wen,LI Meng-tao,ZENG Xiao-feng,ZHANG Feng-chun,DONG Yi..Clinical and prognostic characteristics of 573 cases of primary Sj(o)gren's syndrome[J].中华医学杂志(英文版),2010,123(22):3252-3257,6.

基金项目

This study was supported by grants from Key Projects in the National Science and Technology Pillar Program in the Eleventh Five-Year Plan (No. 2008BAI59B02 and 2008BAI59B03). (No. 2008BAI59B02 and 2008BAI59B03)

中华医学杂志(英文版)

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0366-6999

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