Ribeirão Preto Medical School, Ribeirão Preto, University of São Paulo, Ribeirão Preto, SP, Brazil.
School of Dentistry of Ribeirão Preto, University of São Paulo, Av. do Café, s/n, Ribeirão Preto, SP, 14040-904, Brazil.
Adv Rheumatol. 2022 Jul 1;62(1):23. doi: 10.1186/s42358-022-00255-2.
Sjögren Syndrome (SS) is a systemic autoimmune disease with a wide spectrum of manifestations that can lead to misdiagnosis. This study describes and compares demographic, clinical, serological, and histopathological data from subjects with SS and non-Sjögren Syndrome (NSS). It also details specific features within the primary SS (pSS) and secondary SS (sSS) groups identifying sub-groups.
The sample included individuals referred to an academic medical center in Brazil for investigation of SS from 2012 to 2020. Patients were retrospectively classified as primary SS (pSS), secondary SS (sSS), or NSS, based on the American-European Consensus Group criteria (AECG-2002), after multi-professional clinical and laboratory evaluation.
A total of 676 individuals were screened and 510 (75.4%) completed the assessments; 198 patients were classified as pSS, 149 as sSS, and 163 as NSS. Symptoms and glandular dysfunction tests were similar in the groups. Concerning pSS, extraglandular manifestations were present in 59% of patients; the elderly had more dry symptoms and peripheral neurological disorders; and 2.5% developed non-Hodgkin lymphoma. In sSS, each overlap promoted distinct clinical and laboratory variants. Several alternative diagnoses were identified as a cause of sicca complex in NSS group.
The diagnosis of SS remains a challenge behind dryness. Up to 31% of the suspected cases had other conditions associated to the symptoms. Histopathological analysis of LSG and SSa determined the diagnostic. Aging in pSS and overlap disease in sSS were responsible for distinct phenotypes and characteristic sub-groups in SS.
干燥综合征（SS）是一种全身性自身免疫性疾病，临床表现广泛，可导致误诊。本研究描述并比较了 SS 患者和非干燥综合征（NSS）患者的人口统计学、临床、血清学和组织病理学数据。它还详细说明了原发性 SS（pSS）和继发性 SS（sSS）组中的特定特征，确定了亚组。
该样本包括 2012 年至 2020 年期间因怀疑 SS 而在巴西一家学术医疗中心就诊的个体。根据美国欧洲共识组标准（AECG-2002），在多专业临床和实验室评估后，将患者回顾性分类为原发性 SS（pSS）、继发性 SS（sSS）或 NSS。
共筛查了 676 人，其中 510 人（75.4%）完成了评估；198 例患者被诊断为 pSS，149 例为 sSS，163 例为 NSS。三组患者的症状和腺体功能障碍检测结果相似。在 pSS 中，59%的患者有外分泌腺表现；老年人有更多的干燥症状和周围神经病变；2.5%的患者发生非霍奇金淋巴瘤。在 sSS 中，每种重叠都会导致不同的临床和实验室变异。在 NSS 组中，有多个替代诊断被确定为干燥综合征的病因。
SS 的诊断仍然是干燥背后的一个挑战。多达 31%的疑似病例存在与症状相关的其他疾病。LSG 和 SSa 的组织病理学分析决定了诊断。pSS 中的老龄化和 sSS 中的重叠疾病导致了 SS 中不同的表型和特征亚组。
