Gannot G, Lancaster H E, Fox P C
Clinical Investigations Section, Gene Therapy and Therapeutics Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD, USA.
J Rheumatol. 2000 Aug;27(8):1905-9.
To assess changes in symptoms and signs, salivary function, serologic activity, and disease progression in primary Sjogren's syndrome (SS).
Treatment records on 80 patients seen in clinic and diagnosed with primary SS by defined criteria were reviewed. Forty-nine patients were evaluated at least twice a minimum of 5 years (mean 7 years) apart. Salivary flow rates from each of the major salivary glands and laboratory values were obtained. A structured interview with questions pertaining to signs and symptoms of primary SS was given and a physical examination was performed. An additional 26 patients completed a followup questionnaire by mail and their current medical records were obtained for review. For this group, the followup period was a mean of 10 years after their initial evaluation. Five patients were deceased.
The patients seen twice showed relative stability in their salivary measurements and in their serologic values. The subjective sicca symptoms of oral and ocular dryness among the 75 surviving patients remained prominent. Very few individuals developed another connective tissue disease, therefore evolving into secondary SS. Among the 80 patients, 6 cases of B cell lymphoma were recognized during the followup period.
Although it is not a benign condition, primary SS is a very slowly progressing disease without rapid deterioration in salivary function, systemic markers of disease activity, or dramatic changes in symptoms, with the exception of a high incidence of lymphoma.
评估原发性干燥综合征（SS）的症状体征、唾液功能、血清学活性及疾病进展情况。
回顾了80例临床就诊且根据明确标准诊断为原发性SS患者的治疗记录。49例患者至少接受了两次评估，每次间隔至少5年（平均7年）。获取了每个主要唾液腺的唾液流速及实验室检查值。进行了关于原发性SS体征和症状的结构化访谈并进行了体格检查。另外26例患者通过邮件完成了一份随访问卷，并获取了他们当前的病历以供审查。对于该组患者，随访期为初次评估后的平均10年。5例患者已死亡。
接受两次评估的患者唾液测量值和血清学值相对稳定。75例存活患者的口腔和眼部干燥主观症状仍然突出。很少有人发展为另一种结缔组织病，进而演变为继发性SS。在80例患者中，随访期间确诊6例B细胞淋巴瘤。
尽管原发性SS并非良性疾病，但它是一种进展非常缓慢的疾病，唾液功能、疾病活动的全身标志物或症状均无快速恶化，只是淋巴瘤发病率较高。
