患有全身性硬化症和sicca表现的患者的整体表型:与其他原因引起的sicca表现相比较
François Zimmermann1, François Robin2, Elisabeth Diot3
1Department of Internal Medicine, CHU Rennes, University of Rennes 1, Rennes, France; Université de Rennes, CHU Rennes, Inserm, EHESP, IRSET (Institut de Recherche en Santé, Environnement et Travail) - UMR_S 1085, Rennes, France.
Seminars in arthritis and rheumatism
|July 9, 2024
概括
系统性硬化症患者有Sicca症状显示出显著的唾液腺异常. 与Sjogren病的重叠加剧了这些特征,表明对腺体生理学的特定影响.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 系统性硬化症 (SSc) 是一种自身免疫性疾病,其特征是纤维化和血管异常.
- 西卡症状,粘膜干燥,在SSc患者中很常见.
- 区分SSc相关的sicca症状与其他原因,如Sjogren病 (SjD) 对于准确的诊断和管理至关重要.
研究的目的:
- 为了全面地描述系统性硬化症患者的唾液腺表型,有Sicca症状.
- 为了将这些发现与Sjogren病患者和其他病因症状的患者进行比较.
- 调查潜临床唾液腺参与SSc.
主要方法:
- 招募了60名患有Sicca症状的SSc患者,15名没有Sicca症状的SSc患者和65名患有其他原因的Sicca症状的患者 (包括37名SjD患者).
- 使用主要唾液腺超声波 (SGUS) 和小唾液腺活检 (mSGB) 进行客观评估.
- 相关的临床,组织学和超声波发现.
主要成果:
- 患有西卡症状的SSc患者经常表现出客观的临床,组织学 (44%) 和超声波 (63%) 异常.
- 在没有主观Sicca症状的SSc患者中,超过50%的患者表现出客观的唾液腺异常,表明亚临床参与.
- 与孤立的SjD (48.6%) 和Sicca-SSc (38%) 患者相比,SjD-SSc重叠患者表现出更严重的腺体参与 (70% OMERACT ≥2).
结论:
- 系统性硬化症显著影响唾液腺生理学,导致Sicca特征.
- 斯约格伦病和全身性硬化症的同时发生导致更严重的唾液腺干扰.
- 需要对潜在途径进行进一步的研究,以确定潜在的治疗点.
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