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唾液腺超声对疑似和明确原发性干燥综合征基于症状的表型的鉴别能力。

Discriminative power of salivary gland ultrasound in relation to symptom-based endotypes in suspected and definite primary Sjögren's Syndrome.

机构信息

Department of Internal Medicine and Pediatrics, Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent 9000, Belgium; Faculty of Medicine and Health Sciences, Department of Internal Medicine and Pediatrics, Universiteit Gent, Sint-Pietersnieuwstraat 25, Ghent 9000, Belgium; Center for Inflammation Research, VIB-UGent, Rijvisschestraat 71, Zwijnaarde, 9052, Belgium.

Department of Internal Medicine and Pediatrics, Department of Rheumatology, Ghent University Hospital, C. Heymanslaan 10, Ghent 9000, Belgium; Center for Inflammation Research, VIB-UGent, Rijvisschestraat 71, Zwijnaarde, 9052, Belgium.

出版信息

Semin Arthritis Rheum. 2022 Oct;56:152075. doi: 10.1016/j.semarthrit.2022.152075. Epub 2022 Jul 22.

Abstract

OBJECTIVES

Salivary gland ultrasound (SGUS) is emerging as essential tool in primary Sjögren's Syndrome (pSS), but its link to symptom-based endotypes is unknown. Therefore, we explored SGUS outcomes in relation to endotypes in patients with definite and suspected pSS.

METHODS

Definite pSS patients (n = 171) fulfilling the 2016 ACR/EULAR classification criteria, and suspected pSS patients (n = 119), positive for at least one criterion, were included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT). Stratification into endotypes according to the Newcastle Sjögren's Stratification Tool resulted in low symptom burden (LSB), pain dominant with fatigue (PDF), dryness dominant with fatigue (DDF) and high symptom burden (HSB). SGUS was assessed with Hocevar score (0-48). The dataset was randomly divided into a discovery (n = 203) and replication (n = 87) cohort.

RESULTS

SGUS had strong discriminative power for pSS classification (AUC=0.74), especially in DDF (AUC=0.89). In definite pSS, Hocevar scores in DDF were high compared to other endotypes (38 (20-44) versus 18 (9-33); p < 0.001). Patients with highest SGUS-scores showed more sicca and laboratory abnormalities. Moreover, a subset of young, anti-SSA/Ro positive patients not fulfilling classification criteria showed clear SGUS abnormalities. Replication showed similar results.

CONCLUSIONS

SGUS-scores were significantly higher in definite pSS with DDF endotype, providing the first evidence of imaging abnormalities in salivary glands matching distinct biological profiles ascribed to pSS endotypes. Additionally, a subset of patients with potential early disease was detected based on presence of anti-SSA antibodies and high SGUS-scores. These results underscore the role of SGUS as powerful tool both in pSS classification and stratification.

摘要

目的

唾液腺超声(SGUS)在原发性干燥综合征(pSS)中已成为一种重要的工具,但它与基于症状的表型之间的联系尚不清楚。因此,我们探索了在明确和疑似 pSS 患者中,SGUS 结果与表型之间的关系。

方法

本研究纳入了满足 2016 年 ACR/EULAR 分类标准的明确 pSS 患者(n=171)和至少符合一项标准的疑似 pSS 患者(n=119),这些患者均来自比利时干燥综合征转化试验(BeSSTT)。根据纽卡斯尔干燥综合征分层工具(Newcastle Sjögren's Stratification Tool)进行表型分层,结果分为低症状负担(LSB)、疲劳伴疼痛主导(PDF)、疲劳伴干燥主导(DDF)和高症状负担(HSB)。SGUS 采用 Hocevar 评分(0-48)进行评估。数据集随机分为发现队列(n=203)和验证队列(n=87)。

结果

SGUS 对 pSS 分类具有很强的判别能力(AUC=0.74),特别是在 DDF 中(AUC=0.89)。在明确的 pSS 中,与其他表型相比,DDF 中的 Hocevar 评分较高(38(20-44)比 18(9-33);p<0.001)。SGUS 评分最高的患者出现更多的干燥和实验室异常。此外,一部分年轻、抗 SSA/Ro 阳性且不符合分类标准的患者出现了明显的 SGUS 异常。验证队列也得到了类似的结果。

结论

在 DDF 表型的明确 pSS 中,SGUS 评分明显更高,首次提供了与 pSS 表型相关的唾液腺影像学异常与明确的生物学特征相匹配的证据。此外,还基于存在抗 SSA 抗体和高 SGUS 评分检测到了一部分有潜在早期疾病的患者。这些结果突出了 SGUS 在 pSS 分类和分层中的重要作用。

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