Hospital General de Agudos Bernardino Rivadavia, Ciudad de Buenos Aires, Argentina.
Hospital Alemán, Ciudad de Buenos Aires, Argentina.
Adv Rheumatol. 2022 Jun 7;62(1):19. doi: 10.1186/s42358-022-00250-7.
Primary Sjögren syndrome (pSS) is a chronic autoimmune disease with its main target being exocrine glands, and is the connective tissue disease more frequently associated with other autoimmune diseases. The aim of this study was to assess the frequency of another autoimmune rheumatic disease (ARD) developed in primary Sjögren syndrome (pSS) patients and to describe it's clinical, serological and histologic characteristics.
This is a retrospective cohort study. Data of patients with pSS diagnosis (American-European criteria 2002), included in the GESSAR database (Grupo de Estudio Síndrome de Sjögren, Sociedad Argentina de Reumatología) were analyzed. The development of a second ARD was registered during the follow up.
681 patients were included, 94.8% female. The mean age was 54 (SD 14) years and mean age at diagnosis of 50 (SD 13) years. The mean follow-up was 4.7 (SD 4.9) years; 30 patients (4.41%, CI 95%: 3.1-5.7) developed a second ARD during the follow up, incidence rate was 9.1/1000 patients-year (IR 95%: 5.8-12.4/1000 patients-year), the most frequent being rheumatoid arthritis (RA). 96% out of these 30 patients had xerophthalmia, 86.2% xerostomia, 92% positive Schirmer test, 88.24% positive Rosa Bengala test, lisamine green or Ocular Staining Score, 81.2% positive unstimulated salivary flow, 82.1% Ro(+) and 33.33% La(+). Minor salivary gland biopsy had been performed in 14 of the 30 patients, 12 with positive results. There were no statistically significant differences respect baseline characteristics when comparing the patients who developed another ARD to the ones that did not.
Of all the patients analyzed, 4.4% presented another ARD during their follow-up. It is important to be aware of this, to make an early and proper diagnosis and treatment of our patients.
原发性干燥综合征（pSS）是一种以外分泌腺为主要靶器官的慢性自身免疫性疾病，也是与其他自身免疫性疾病关系更为密切的结缔组织病。本研究旨在评估原发性干燥综合征（pSS）患者中另一种自身免疫性风湿病（ARD）的发生频率，并描述其临床、血清学和组织学特征。
这是一项回顾性队列研究。分析了包含在 GESSAR 数据库（Grupo de Estudio Síndrome de Sjögren，Sociedad Argentina de Reumatología）中的符合美国欧洲 2002 年干燥综合征分类标准的 pSS 患者的数据。在随访期间记录了第二种 ARD 的发生情况。
共纳入 681 例患者，94.8%为女性。平均年龄为 54（14）岁，诊断时平均年龄为 50（13）岁。平均随访时间为 4.7（4.9）年；30 例（4.41%，95%可信区间：3.1-5.7）在随访期间发生了第二种 ARD，发病率为 9.1/1000 患者年（95%可信区间：5.8-12.4/1000 患者年），最常见的是类风湿关节炎（RA）。这 30 例患者中有 96%出现干眼症，86.2%出现口干症，92%Schirmer 试验阳性，88.24%Rosa Bengala 试验阳性、泪液滤纸试验或眼部染色评分阳性，81.2%唾液分泌量非刺激试验阳性，82.1%Ro（+）和 33.33%La（+）。30 例患者中有 14 例进行了唇腺活检，结果均为阳性。与未发生第二种 ARD 的患者相比，发生第二种 ARD 的患者在基线特征方面无统计学差异。
在所有分析的患者中，4.4%在随访期间出现了另一种 ARD。因此，我们需要提高对这种情况的认识，以便对患者进行早期、恰当的诊断和治疗。
