Department of Rheumatology and Internal Medicine, Medical University of Wroclaw, Borowska 213, 50-556, Wroclaw, Poland.
Department of General, Minimally Invasive and Endocrine Surgery, Medical University of Wroclaw, Wroclaw, Poland.
Rheumatol Int. 2020 Mar;40(3):399-404. doi: 10.1007/s00296-020-04522-7. Epub 2020 Feb 1.
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.
淋巴瘤是原发性干燥综合征（pSS）的严重并发症之一。本研究旨在评估 pSS 患者中淋巴瘤的发生频率。这项单中心回顾性研究纳入了 198 名符合 pSS 诊断标准的白种人患者。根据 2016 年 WHO 分类，对淋巴增生性疾病的类型进行了分类。在 pSS 诊断后，观察的平均时间为 48 周。85%的患者存在焦点评分（FS）≥1，84%的患者检测到抗 SSA 抗体。130 名（65%）患者检测到类风湿因子。根据 EULAR 干燥综合征疾病活动指数（ESSDAI），pSS 诊断时的平均疾病活动指数为 8.3 分。14%的患者补体 C3 降低，10%的患者补体 C4 降低。4 名患者（2%）被诊断为淋巴瘤。大多数患者被诊断为黏膜相关淋巴组织淋巴瘤（MALT），肿瘤位于腮腺，1 例患者胃受累。最后，1 例患者被诊断为罕见的肺 B 细胞小淋巴细胞淋巴瘤。在本文中，我们详细介绍了每个病例的特征。在分析人群中，pSS 患者在 pSS 病程中发生淋巴瘤的频率为 2%。pSS 患者的淋巴瘤类型多样，这要求在每次就诊时对每个患者的疾病活动进行个体化监测。
