Department of Internal Medicine, Limoges University Hospital, Limoges, France.
J Clin Immunol. 2011 Oct;31(5):840-7. doi: 10.1007/s10875-011-9553-3. Epub 2011 Jul 9.
The aim of this paper was to study the evolution of primary Sjögren's syndrome (pSS) immunological profile, its impact on pSS activity and long-term evolution in a bicentric cohort of French patients with pSS (n = 445, mean age 53.6 ± 14 years, mean follow-up 76.1 ± 51 months).
This is a retrospective cohort study.
Two hundred twelve patients were Sjögren's syndrome A (SSA) positive, and 131 were both SSA and Sjögren's syndrome B (SSB) positive. Sixty-eight patients (15%) had cryoglobulinemia. Active systemic profile (i.e., hypergammaglobulinemia, rheumatoid factor (RF), and anti-Sjögren's syndrome A (anti-SSA), anti-Sjögren's syndrome B (anti-SSB) positivity), associated with multisystemic involvement, leads to an increased utilization of corticosteroid and hydroxychloroquine. Multivariate analysis pointed out independent statistical association between hypergammaglobulinemia, anti-SSA, anti-SSB, and RF. Cryoglobulinemia is associated with multi-systemic involvement, lymphoma, and pSS-related death.
The subset of patients with active immunological profile is characterized by systemic complications leading to immunosuppressive drug utilization and polyclonal B-cell activation profile.
本文旨在研究原发性干燥综合征（pSS）免疫特征的演变，及其对 pSS 活动性和长期演变的影响，该研究纳入了来自法国的 pSS 患者（n=445，平均年龄 53.6±14 岁，平均随访时间 76.1±51 个月）的两个中心队列。
这是一项回顾性队列研究。
212 例患者抗 Sjögren 综合征 A（SSA）阳性，131 例患者 SSA 和 Sjögren 综合征 B（SSB）均阳性。68 例（15%）患者存在冷球蛋白血症。活跃的全身性特征（即高丙种球蛋白血症、类风湿因子（RF）、抗 Sjögren 综合征 A（抗-SSA）、抗 Sjögren 综合征 B（抗-SSB）阳性），伴有多系统受累，导致皮质类固醇和羟氯喹的使用增加。多变量分析指出高丙种球蛋白血症、抗-SSA、抗-SSB 和 RF 之间存在独立的统计学关联。冷球蛋白血症与多系统受累、淋巴瘤和 pSS 相关死亡有关。
具有活跃免疫特征的患者亚群的特点是存在系统性并发症，导致免疫抑制药物的使用和多克隆 B 细胞激活。
