Yazisiz Veli, Aslan Bengisu, Erbasan Funda, Uçar İsmail, Öğüt Tahir Saygin, Terzioğlu Mustafa Ender
The Department of Internal Medicine, The Division of Rheumatology, Akdeniz University Medical School, Antalya, Turkey.
Clin Rheumatol. 2021 Jan;40(1):221-229. doi: 10.1007/s10067-020-05154-9. Epub 2020 Jun 5.
This study compared the clinical and serological characteristics of seronegative and seropositive primary Sjögren syndrome (pSS) and examined whether current classification criteria for pSS cover seronegative pSS.
The study group comprised 375 patients (341 women and 34 men) diagnosed with pSS. A clinical diagnosis by an expert rheumatologist was considered the "gold standard" for the diagnosis of pSS. The clinical and serological characteristics of the patients were retrospectively collected from hospital medical files.
Fifty-eight of the 375 pSS patients (15.5%) were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. There were no statistically significant differences in terms of patient age, age at diagnosis, sex distribution, clinical features, and laboratory findings between seronegative and seropositive pSS. The frequency of hypergammaglobulinemia was higher in seropositive pSS. The 2016 ACR/ULAR criteria best covered most seronegative pSS cases (84.5%). For seronegative pSS, the agreement between the 2002 AECG, 2012 ACR, and 2016 ACR/EULAR criteria was relatively low.
The clinical features of seronegative pSS (i.e., a lack of four autoantibodies in serum) were similar to those of seropositive pSS. The current classification criteria for pSS should not be used in the diagnosis of seronegative pSS, as the agreement between the different sets of criteria was low, and some patients fell outside the classification. Further clinical and laboratory studies are needed to identify the features that distinguish seronegative pSS. Key Points • Approximately 15% of the pSS patients were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. • Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. • The clinical features of seronegative pSS were similar to those of seropositive pSS. • The current classification criteria for pSS should not be used in the diagnosis of seronegative patients, as the agreement between the different sets of criteria was low, and some patients fell outside the classification.
本研究比较了血清阴性和血清阳性原发性干燥综合征（pSS）的临床和血清学特征，并探讨了当前pSS的分类标准是否涵盖血清阴性pSS。
研究组包括375例诊断为pSS的患者（341名女性和34名男性）。由风湿科专家进行的临床诊断被视为pSS诊断的“金标准”。患者的临床和血清学特征通过回顾医院病历收集。
375例pSS患者中有58例（15.5%）ANA、RF、抗Ro和抗La自身抗体血清学阴性。血清阴性pSS根据唇活检样本中的淋巴细胞浸润进行诊断。血清阴性和血清阳性pSS在患者年龄、诊断年龄、性别分布、临床特征和实验室检查结果方面无统计学显著差异。血清阳性pSS中高球蛋白血症的发生率更高。2016年美国风湿病学会/欧洲抗风湿病联盟（ACR/ULAR）标准能最好地涵盖大多数血清阴性pSS病例（84.5%）。对于血清阴性pSS，2002年美国欧洲共识小组（AECG）、2012年ACR和2016年ACR/EULAR标准之间的一致性相对较低。
血清阴性pSS（即血清中缺乏四种自身抗体）的临床特征与血清阳性pSS相似。当前pSS的分类标准不应应用于血清阴性pSS的诊断，因为不同标准集之间的一致性较低，且一些患者不在分类范围内。需要进一步的临床和实验室研究来确定区分血清阴性pSS的特征。要点 • 约15%的pSS患者ANA、RF、抗Ro和抗La自身抗体血清学阴性。 • 血清阴性pSS根据唇活检样本中的淋巴细胞浸润进行诊断。 • 血清阴性pSS的临床特征与血清阳性pSS相似。 • 当前pSS的分类标准不应应用于血清阴性患者的诊断，因为不同标准集之间的一致性较低，且一些患者不在分类范围内。
