Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Semin Arthritis Rheum. 2013 Feb;42(4):368-76. doi: 10.1016/j.semarthrit.2012.07.003. Epub 2012 Sep 18.
To determine whether the presence of germinal centers (GCs) in salivary glands of patients with primary Sjögren's syndrome (pSS) is related to the severity of disease course and distinct immunopathology features.
A systematic search was performed in September 2011 for terms and synonyms of Sjögren's syndrome and germinal centers. A total of 80 articles were retrieved, of which 16 were included for (meta-) analysis.
GC morphology was present in a mean ± SD 25.1 ± 5.0% of pSS patients. Mean lymphocyte focus scores were 1.25 points higher in patients with GCs as compared to those without GCs. Saliva production was reduced in patients with GCs, although this did not reach statistical significance. Percentages of patients positive for rheumatoid factor, anti-Sjögren's syndrome A (SSA), and anti-Sjögren's syndrome B (SSB) antibodies were significantly higher in patients with GCs (mean increase, 15%, 18%, and 18%, respectively). Additionally, patients with GCs were characterized by enhanced levels of local and systemic proinflammatory mediators. Importantly, these patients have a higher risk of lymphoma development (14% versus 1%).
Patients with GCs are characterized by more severe disease, although the small number of studies and their design hamper generalizability of results. The precise mechanisms that contribute to the development and persistence of germinal centers in pSS are largely unknown. This and the strongly increased risk of lymphoma development warrant intensive studies for the role of germinal centers in the immunopathology of pSS.
确定原发性干燥综合征（pSS）患者唾液腺中是否存在生发中心（GC）与疾病严重程度和独特的免疫病理学特征之间的关系。
于 2011 年 9 月，采用干燥综合征和生发中心相关术语和同义词进行系统检索。共检索到 80 篇文章，其中 16 篇文章进行（meta-）分析。
GC 形态存在于 pSS 患者中的平均 ± SD 为 25.1 ± 5.0%。与无 GC 的患者相比，有 GC 的患者的淋巴细胞灶评分平均高 1.25 分。尽管唾液产生减少，但未达到统计学意义。有 GC 的患者类风湿因子、抗干燥综合征 A（SSA）和抗干燥综合征 B（SSB）抗体阳性的患者比例明显高于无 GC 的患者（分别平均增加 15%、18%和 18%）。此外，有 GC 的患者表现为局部和全身促炎介质水平升高。重要的是，这些患者发生淋巴瘤的风险更高（14%比 1%）。
尽管研究数量少且设计存在局限性，但有 GC 的患者具有更严重的疾病。生发中心在 pSS 中的发展和持续存在的确切机制尚不清楚。这与淋巴瘤发生风险的显著增加，提示需要深入研究生发中心在 pSS 免疫病理学中的作用。
