Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Greece.
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.
Clin Exp Rheumatol. 2022 Dec;40(12):2298-2302. doi: 10.55563/clinexprheumatol/gqvyus. Epub 2022 Oct 5.
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by oral and eye dryness. A minority of patients can present without dryness but studies on their clinico-laboratory manifestations are scarce. Our purpose was to describe the clinical phenotype of pSS patients lacking sicca symptoms.
From a total of 1738 consecutive pSS patients fulfilling the 2016 ACR-EULAR criteria, those who presented without sicca symptoms were identified (non-dryness group). Their medical data was collected and compared with 2 control groups: a) the remaining unmatched sicca pSS patients with both oral and eye dryness (unmatched dryness group) and b) matched sicca pSS patients according to age, sex, and disease duration, in 1:2 ratio (matched dryness group).
Thirty-eight (2.19%) patients lacked sicca manifestations presenting mainly with arthralgias (47%), parotid enlargement (24%), Raynaud's phenomenon (11%) and persistent lymphadenopathy (11%) that led them to be evaluated for pSS. Non-dryness pSS patients were younger than the unmatched sicca controls, displaying a higher frequency of anti-Ro/SSA antibodies (100% vs. 79.7%, p<0.001), ANA positivity (100% vs. 90.4%, p<0.001), neutropenia (20.8% vs. 7.5%, p=0.04) and thrombocytopenia (13.8% vs. 4.2%, p=0.04). They also had lower frequency of positive ocular tests compared to both unmatched and matched dryness patients. No differences were found between non-dryness pSS patients and both control groups regarding focus score or any other extraglandular manifestation.
pSS patients without sicca complaints constitute a distinct phenotype involving younger patients, sharing common immunopathologic mechanisms with typical sicca patients.
原发性干燥综合征（pSS）是一种以口干和眼干为特征的系统性自身免疫性疾病。少数患者可无干燥症状，但有关其临床-实验室表现的研究甚少。本研究旨在描述无干燥症状的 pSS 患者的临床表型。
共纳入 1738 例符合 2016 年 ACR-EULAR 标准的连续 pSS 患者，其中识别出无干燥症状的患者（无干燥组）。收集他们的临床资料，并与 2 个对照组进行比较：a）其余不伴有口干和眼干的未匹配的干燥 pSS 患者（未匹配的干燥组）和 b）按年龄、性别和疾病持续时间以 1:2 比例匹配的干燥 pSS 患者（匹配的干燥组）。
38 例（2.19%）患者无干燥表现，主要表现为关节痛（47%）、腮腺肿大（24%）、雷诺现象（11%）和持续性淋巴结病（11%），这些症状促使他们被评估为 pSS。与未匹配的干燥对照组相比，无干燥 pSS 患者更年轻，抗 Ro/SSA 抗体阳性率更高（100% vs. 79.7%，p<0.001）、ANA 阳性率更高（100% vs. 90.4%，p<0.001）、中性粒细胞减少症发生率更高（20.8% vs. 7.5%，p=0.04）和血小板减少症发生率更高（13.8% vs. 4.2%，p=0.04）。与未匹配和匹配的干燥组患者相比，他们的眼部检查阳性率也较低。无干燥 pSS 患者与两个对照组之间在焦点评分或任何其他外分泌腺表现方面均无差异。
无口干症状的 pSS 患者构成了一个独特的表型，涉及年轻患者，与典型干燥患者具有共同的免疫病理机制。
