Lymphomas are one of the serious complications of the primary Sjörgen's Syndrome (pSS). The aim of the study was to evaluate the frequency of lymphoma in pSS. The singe-center retrospective study included 198 Caucasian patients, who met diagnostic criteria for pSS. The type of lymphoproliferative disorder was classified according to the WHO 2016 classification. The mean time of observation, after pSS diagnosis, was 48 weeks. Focus score (FS) ≥ 1 was present in 85% of the patients, and anti-SSA antibodies were detected in 84%. Rheumatoid factor was detected in 130 (65%) patients. Mean disease activity index, according to EULAR Sjörgen's Syndrome disease activity index (ESSDAI), was 8.3 points at the moment of pSS diagnosis. Complement C3 was decreased in 14% of the patients, while 10% showed reduced complement C4. Four patients (2%) were diagnosed with a lymphoma. Most of the patients were diagnosed with mucosa-associated lymphoid tissue lymphoma (MALT), in whom the tumour was located in the parotid gland, and in one patient the stomach was involved. Finally, one patient was diagnosed with a rare B-cell small lymphocytic lymphoma located in the lungs. In this article, we present detailed characteristics of each case. In analysed population the frequency of lymphoma in the course of pSS in patients with pSS is 2%. The variety of lymphoma types in pSS patients imposes individual monitoring in each patient at every check-up visit for disease activity.

中文翻译：

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原发性干燥综合征患者的淋巴细胞增生性疾病的患病率和临床表现。

淋巴瘤是原发性舍尔根氏综合症（pSS）的严重并发症之一。该研究的目的是评估pSS中淋巴瘤的发生率。以单中心为中心的回顾性研究包括198名符合pSS诊断标准的白人患者。淋巴增生性疾病的类型根据WHO 2016分类进行分类。经pSS诊断后，平均观察时间为48周。在85％的患者中存在焦点得分（FS）≥1，在84％的患者中检测到抗SSA抗体。在130（65％）位患者中检测到类风湿因子。根据EULARSjörgen的综合症疾病活动指数（ESSDAI），平均疾病活动指数在pSS诊断时为8.3点。14％的患者补体C3降低，而10％的患者补体C4降低。四名患者（占2％）被诊断患有淋巴瘤。大多数患者被诊断患有粘膜相关淋巴样组织淋巴瘤（MALT），肿瘤位于腮腺中，其中一名患者胃部受累。最后，一名患者被诊断出患有罕见的位于肺部的B细胞小淋巴细胞淋巴瘤。在本文中，我们介绍了每种情况的详细特征。在所分析的人群中，pSS患者中pSS过程中淋巴瘤的发生率为2％。pSS患者中各种类型的淋巴瘤在每次检查就诊时都对每位患者进行疾病活动的单独监测。最后，一名患者被诊断出患有罕见的位于肺部的B细胞小淋巴细胞淋巴瘤。在本文中，我们介绍了每种情况的详细特征。在所分析的人群中，pSS患者中pSS过程中淋巴瘤的发生率为2％。pSS患者中各种类型的淋巴瘤在每次检查就诊时都对每位患者进行疾病活动的单独监测。最后，一名患者被诊断出患有罕见的位于肺部的B细胞小淋巴细胞淋巴瘤。在本文中，我们介绍了每种情况的详细特征。在所分析的人群中，pSS患者中pSS过程中淋巴瘤的发生率为2％。pSS患者中各种类型的淋巴瘤在每次检查就诊时都对每位患者进行疾病活动的单独监测。