Fragkioudaki Sofia, Mavragani Clio P, Moutsopoulos Haralampos M
Department of Physiology Department of Pathophysiology Joint Academic Rheumatology Program, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Medicine (Baltimore). 2016 Jun;95(25):e3766. doi: 10.1097/MD.0000000000003766.
The heightened risk of non-Hodgkin lymphoma (NHL) development in primary Sjogren syndrome (SS) is well established. Several adverse clinical and laboratory predictors have been described. In the current work, we aimed to formulate a predictive score for NHL development, based on clinical, serological, and histopathological findings at the time of SS diagnosis. In the present case-control study of 381 primary SS patients and 92 primary SS patients with concomitant NHL, clinical, serological, and histopathological variables at the time of SS diagnosis were retrospectively recorded. For the identification of predictors for NHL development univariate and multivariate models were constructed. Salivary gland enlargement (SGE), lymphadenopathy, Raynaud phenomenon, anti-Ro/SSA or/and anti-La/SSB autoantibodies, rheumatoid factor (RF) positivity, monoclonal gammopathy, and C4 hypocomplementemia were shown to be independent predictors for NHL development. On the basis of the number of independent risk factors identified, a predictive risk score for NHL development was formulated. Thus, patients presenting with ≤2 risk factors had a 3.8% probability of NHL development, those with 3 to 6 risk factors 39.9% (OR (95%CI): 16.6 [6.5-42.5], P < 0.05), while in the presence of all 7 risk factors the corresponding probability reached 100% (OR [95%CI]: 210.0 [10.0-4412.9], P < 0.0001). In conclusion, an easy to use diagnostic scoring tool for NHL development in the context of SS is presented. This model is highly significant for the design of early therapeutic interventions in high risk SS patients for NHL development.
原发性干燥综合征（SS）患者发生非霍奇金淋巴瘤（NHL）的风险增加已得到充分证实。已有多项不良临床和实验室预测指标被描述。在当前研究中，我们旨在根据SS诊断时的临床、血清学和组织病理学结果，制定一个NHL发生的预测评分系统。在这项针对381例原发性SS患者和92例合并NHL的原发性SS患者的病例对照研究中，回顾性记录了SS诊断时的临床、血清学和组织病理学变量。为了确定NHL发生的预测指标，构建了单变量和多变量模型。唾液腺肿大（SGE）、淋巴结病、雷诺现象、抗Ro/SSA或/和抗La/SSB自身抗体、类风湿因子（RF）阳性、单克隆丙种球蛋白病和C4低补体血症被证明是NHL发生的独立预测指标。根据确定的独立危险因素数量，制定了NHL发生的预测风险评分。因此，存在≤2个危险因素的患者发生NHL的概率为3.8%，存在3至6个危险因素的患者为39.9%（OR（95%CI）：16.6 [6.5 - 42.5]，P<0.05），而存在所有7个危险因素时，相应概率达到100%（OR [95%CI]：210.0 [10.0 - 4412.9]，P<0.0001）。总之，我们提出了一种在SS背景下易于使用的NHL发生诊断评分工具。该模型对于设计针对NHL发生风险较高的SS患者的早期治疗干预措施具有高度重要性。
