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Primary Sj(o|¨)gren’s Syndrome in China:Clinical and Immunologic Expression in640Patients

Author: LiZuo
Tutor: ZhangFengChun
School: Beijing Union Medical College
Course: Internal Medicine
Keywords: primary sjogren’s syndrome current study clinical feature
CLC: R593
Type: PhD thesis
Year: 2013
Downloads: 43
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Abstract


Objective We conducted the study to establish a nationwide patient registration database platform of primary sjogren’s syndrome (pSS), characterize the clinical presentation of pSS in a large cohort of Chinese patients, determine whether epidemiologic, clinical, and analytical features modulate disease expression, analyse factors affecting patient’s first treatment and final diagnosis, and compare those data with the domestic and international research groups. From this study we could get a better understanding of the disease status of Chinese pSS patients, and improve the level of diagnosis and treatment of pSS.Methods A total of640patients with pSS according to the2002revised American European classification criteria from16clinical centers in China were enrolled in the study between January2009and November2011. Patient’s registration database platform was established. Patient’s epidemiology profile and cumulative clinical and immunologic data were collected. Patients were divided into different subgroups according to their epidemiology feature, course and severity of disease, and immunologic profile.T-test, nonparametric test and Chi-square test were applied to compare data in two groups. Regression analysis and multivariate linear regression analysis models were used to do multivariate analysis.Results1. Characteristics of Chinese pSS patients:95.3%of the patients were female. Mean age of onset was44.6±12.0. The most common symptom of pSS were dry mouth(90.9%), and dry eye (72.3%).91.3%of pSS patients had systemic involvement, which mainly were blood system(45.8%), lung(44.3%), joints (40.9%), kidney (33.0%) and liver (27.6%).The positive rates of objective tests of salivary gland, lacrimal gland and salivary gland biopsy were87.6%,97.1%and86.3%. The positive rates of ANA, anti SSA, anti SSB and RF were78.4%,51.5%,27.2%and64.6%.2. Characteristics of different subgroups:①4.7%of pSS patients were male. Compared with female, they had older average age of onset, higher positive rates of liver involvement, chronic renal insufficiency, and increased IgM.②The age of onset of10.8%of pSS patients was less than30. Those patients had lower incidence rates of arthritis and interstitial lung disease (ILD) than patients with age of onset30-59.10.8%of pSS patients had age of onset more than60. Compared with patients with age of onset30-59, those patients had less female, shorter period for first treatment and final diagnosis, higher incidence rates of dry mouth and ILD, and lower positive rates of parotid gland enlargement, blood WBC decrease, anti SSA and SSB. Compared with patients with age of onset<30, patients with age of onset≥60had higher incidence rates of dry mouth, liver dysfunction and ILD, lower positive rates of blood WBC decrease, anti SSA and SSB.③The course of disease of14.1%patients was more than10years. Those patients had lower age of onset, longer periods for first treatment and final diagnosis, higher positive rates of parotid gland enlargement, fever and blood WBC decrease, lower positive rate of salivary gland injury.④8%of patients’disease limited in exocrine gland.Those patients had lower positive rate of eye tests.⑤28.8%of patients had ILD. Those patients had older age of onset, higher positive rates of dry mouth, objective tests of salivary gland injury, liver dysfunction,ANA≥1:80and serum IgA increase, and lower positive rates of anti SSA and SSB. Multi factor analysis revealed ANA≥1:80and serum IgA increase were correlative factors of ILD.⑥43.1%of patients had ANA titer≥1:320. Those patients had longer periods for first treatment and final diagnosis, lower positive rates of objective salivary gland injury tests and proteinuria, and higher incidence rates of labial gland biopsy, and RF.⑦52%of patients had positive anti SSA. Those patients had younger age of onset, lower positive rates of dry mouth, dry eye, labial gland pathology, ILD, and RF. And had higher positive rates of patriod gland enlargement, fatigue, proteinuria, blood WBC decrease and anti SSB.⑧27%of patients had positive anti SSB. Those patients had younger age of onset, lower positive rate of ILD, and higher rates of proteinuria, blood WBC decrease, anti SSA and decreased C4.⑨64.6%of patients had positive RF. Those patients had lower rates of PLT decrease and positive anti SSA, and higher positive rate of ANA≥1:320.⑩85.3%of patients had positive anti SSA, anti SSB, ANA≥1:320and/or RF. Those patients had younger age of onset, higher incidence rates of blood WBC decrease and proteinuria, and lower positive rate of labial gland pathology.(11)86.3%of patients had positive labial gland pathology. Those patients had higher rate of ANA≥1:320, while the positive rate of anti SSA was lower than other patients.3. The median period between onset of symptom and first treatment was15 months. Between onset of symptom and final diagnosis was21months. Multiple linear regression showed the factor effected those periods was with purpura as initial symptom.4. Logistic regression analysis showed those with dry mouth were5.5times more likely to have dry eye than no dry eye, and0.2times more likely to have positive anti SSA/SSB than negative anti SSA/SSB. Those with dry eye were5.5times more likely to have dry mouth than no dry mouth, and the patients with anti SSA/SSB positive were0.2times more likely to have dry mouth than no dry mouth, while controlling for other phenotypic features of SS.5. Compared with European studies, this study found that the age of onset was10years younger than European patients. The positive rates of dry mouth and dry eye were lower, and systemic involvement was higher than European patients.Conclusion The majority of pSS patients in China were female, and the age of onset was younger than European patients. The outstanding performance of pSS was dry mouth and dry eye, while systemic involvement was very common. Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of pSS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers.Those findings were conducive to the early estimate of disease prognosis and outcome, and make individualized treatment plan.

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