抗 Ro/SS-A 和抗 La/SS-B 阴性原发性干燥综合征的临床和血清学特征：一项对比研究。

Clinical and serological characteristics of anti-Ro/SS-A and anti-La/SS-B negative primary Sjögren's syndrome: a comparative study.

机构信息

Department of Internal Medicine, Division of Rheumatology, Eskisehir City Hospital, Eskisehir, Turkey.

出版信息

Eur Rev Med Pharmacol Sci. 2024 Mar;28(5):1760-1767. doi: 10.26355/eurrev_202403_35589.

DOI:10.26355/eurrev_202403_35589

PMID:38497858

Abstract

OBJECTIVE

This study aimed to describe the clinical spectrum of primary Sjögren's syndrome (pSS) patients with anti-Ro/SS-A and anti-La/SS-B negativity.

PATIENTS AND METHODS

From a single-center study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, those with triple seronegativity anti-Ro/SS-A (anti-Sjögren's-syndrome-related antigen A autoantibody), anti-La/SS-B (anti-Sjögren's-syndrome-related antigen B autoantibody), rheumatoid factor (RF) (-) and antinuclear antibody (ANA)(+)] or [anti-Ro/SS-A(-), anti-La/ SS-B(-), RF(+) and ANA(-)] and quad¬ruple seronegativity [anti-Ro/SS-A(-), anti-La/SS-B(-), RF(-) and ANA(-)] were identified retrospectively. Clinical, serological, and laboratory features were compared. A comparison between triple and quadruple seronegative pSS patients was also performed.

RESULTS

We included 184 patients (168 women, 16 men) with a mean age at diagnosis of 50.1±13.1 years. The most common subjective presenting features at the time of the diagnosis were dry mouth (94.5%) and dry eye (91.3 %). ANA positivity was 57.0%, and RF positivity was 30.4%. Salivary gland enlargement, arthritis, Raynaud's phenomenon, vasculitis, interstitial lung disease (ILD), neurological involvement, primary biliary cholangitis (PBC), lymphopenia, and thrombocytopenia were observed in ANA+ and RF+ patients but not in seronegative patients (p<0.0001). Arthritis was observed most frequently in RF-positive patients and secondly in ANA-positive patients, whereas arthritis was not observed in seronegative patients (p<0.0001). Autoimmune thyroiditis was present in 65 patients (35.0%), 84.6% of these patients were ANA positive while 12.3% were ANA negative (p=0.0014), RF positivity was 30.7% while RF negativity was 6.15% (p=0.001), 23.0% were both ANA and RF positive while 12.3% were seronegative (p<0.002). Cryoglobulinemia, renal disease, and lymphoma were not observed in any of the patients.

CONCLUSIONS

We confirm the strong influence of immunological markers on the phenotype of primary SS at diagnosis.

摘要

目的

本研究旨在描述原发性干燥综合征（pSS）患者中抗 Ro/SS-A 和抗 La/SS-B 阴性的临床谱。

方法

从一项单中心连续 SS 患者研究人群中，选取同时满足 2016 年 ACR-EULAR 分类标准、三联阴性（抗 Ro/SS-A[抗 Sjögren 综合征相关抗原 A 自身抗体]、抗 La/SS-B[抗 Sjögren 综合征相关抗原 B 自身抗体]、类风湿因子[RF]（-）和抗核抗体[ANA]（+）]或 [抗 Ro/SS-A（-）、抗 La/SS-B（-）、RF（+）和 ANA（-）]）和四联阴性[抗 Ro/SS-A（-）、抗 La/SS-B（-）、RF（-）和 ANA（-）]的患者。回顾性比较临床、血清学和实验室特征。还比较了三联和四联阴性 pSS 患者之间的差异。

结果

我们纳入了 184 名患者（168 名女性，16 名男性），诊断时的平均年龄为 50.1±13.1 岁。最常见的首发症状是口干（94.5%）和眼干（91.3%）。ANA 阳性率为 57.0%，RF 阳性率为 30.4%。唾液腺肿大、关节炎、雷诺现象、血管炎、间质性肺病（ILD）、神经系统受累、原发性胆汁性胆管炎（PBC）、淋巴细胞减少和血小板减少仅见于 ANA 和 RF 阳性患者，而在血清学阴性患者中未观察到（p<0.0001）。关节炎最常发生在 RF 阳性患者中，其次是 ANA 阳性患者，而血清学阴性患者中未观察到关节炎（p<0.0001）。自身免疫性甲状腺炎见于 65 名患者（35.0%），其中 84.6%的患者 ANA 阳性，而 12.3%的患者 ANA 阴性（p=0.0014），RF 阳性率为 30.7%，RF 阴性率为 6.15%（p=0.001），23.0%的患者同时存在 ANA 和 RF 阳性，而 12.3%的患者为血清学阴性（p<0.002）。任何患者均未观察到冷球蛋白血症、肾脏疾病和淋巴瘤。