Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece.
Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Greece.
Clin Exp Rheumatol. 2022 Dec;40(12):2357-2362. doi: 10.55563/clinexprheumatol/263xbc. Epub 2022 Dec 20.
Previous cohort studies have shown that around 10% of patients with primary Sjögren's syndrome (pSS) develop lymphadenopathy during their disease course. However, no studies have described their clinical phenotype. The present study aims to describe the clinical manifestations and laboratory findings of pSS patients presenting long-standing lymphadenopathy.
From a total of 1234 consecutive pSS patients fulfilling the 2016 ACR-EULAR criteria, those with stable lymphadenopathy unrelated to lymphoma were identified (lymphadenopathy group). Their clinical data were collected and compared with 2 control groups: a) the remaining unmatched pSS patients without lymphadenopathy (unmatched non-lymphadenopathy group) and b) pSS patients without lymphadenopathy matched for age, sex, and disease duration, in an approximately 1:1 ratio (matched non-lymphadenopathy group).
One hundred and sixty-five (13.37%) patients presented persistent, stable lymphadenopathy. They were characterised by younger age at both pSS onset and diagnosis, and by shorter disease duration. Compared to the unmatched nonlymphadenopathy group, patients with lymphadenopathy had more frequently salivary gland enlargement (p<0.001), higher focus score at first salivary gland biopsy (p=0.017), palpable purpura (p<0.001), peripheral nervous system involvement (p=0.012), glomerulonephritis (p<0.001), and leukopenia (p<0.001), while the results of the matched comparison were similar. Regarding the serological profile, the comparison with the unmatched group demonstrated higher frequency of ANA (p=0.013), anti-Ro/SSA (p=0.001), and anti-La/SSB (p<0.001) positivity for the lymphadenopathy group, while in the matched comparison only higher rates of anti-Ro/SSA positivity (p=0.002) remained statistically significant.
pSS patients presenting non-lymphoma related stable lymphadenopathy constitute a subgroup of younger individuals with B-cell hyperactivation.
先前的队列研究表明，约 10%的原发性干燥综合征（pSS）患者在疾病过程中出现淋巴结病。然而，尚无研究描述其临床表型。本研究旨在描述长期存在淋巴结病的 pSS 患者的临床表现和实验室检查结果。
从总共 1234 例符合 2016 年 ACR-EULAR 标准的连续 pSS 患者中，确定了与淋巴瘤无关的稳定淋巴结病患者（淋巴结病组）。收集并比较了他们的临床数据，与 2 个对照组进行比较：a）其余无淋巴结病的未配对 pSS 患者（未配对无淋巴结病组）和 b）年龄、性别和疾病持续时间匹配约 1:1 的无淋巴结病的 pSS 患者（配对无淋巴结病组）。
165 例（13.37%）患者表现为持续、稳定的淋巴结病。这些患者在 pSS 发病和诊断时年龄较小，且疾病持续时间较短。与未配对的无淋巴结病组相比，淋巴结病患者更常出现唾液腺肿大（p<0.001），首次唾液腺活检的焦点评分更高（p=0.017），可触及性紫癜（p<0.001），周围神经系统受累（p=0.012），肾小球肾炎（p<0.001）和白细胞减少症（p<0.001），而匹配比较的结果相似。关于血清学特征，与未配对组的比较显示，淋巴结病组抗核抗体（ANA）（p=0.013）、抗 Ro/SSA（p=0.001）和抗 La/SSB（p<0.001）阳性率更高，而在匹配比较中，只有抗 Ro/SSA 阳性率更高（p=0.002）仍然具有统计学意义。
表现为非淋巴瘤相关稳定淋巴结病的 pSS 患者构成了 B 细胞过度激活的年轻患者亚群。
