稳定UCTD患者的疾病演变和器官损伤累积:长期单心初始队列
Chiara Tani1, Francesca Trentin2, Alice Parma3
1Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy chiara.tani@unipi.it.
RMD open
|April 26, 2024
概括
稳定无差异连接组织疾病 (sUCTD) 可以导致器官损伤,并随着时间的推移演变成定义的连接组织疾病,尽管不如系统性红斑狼 (SLE) 如此频繁. 对sUCTD患者的长期结果需要仔细监测.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 内部医学 内部医学
背景情况:
- 不分化的结缔组织疾病 (UCTD) 是一种缺乏分类标准的系统性自身免疫性疾病.
- 大多数UCTD长期保持不分化 (稳定UCTD,sUCTD).
- 关于sUCTD的长期结果和疾病进展的数据有限.
研究的目的:
- 研究稳定的UCTD患者的长期演变和损伤累积.
- 在sUCTD中识别与疾病进展和器官损伤相关的因素.
- 将sUCTD的长期结果与年龄和性别匹配的系统性红斑狼 (SLE) 患者的长期结果进行比较.
主要方法:
- 对141名sUCTD患者的初始队列进行了回顾性纵向分析.
- 评估疾病演变和损害累积在1,5年和10年.
- 使用了部分最小方形 (PLS) 回归和尼尔森-阿伦分析来与SLE队列进行结果比较.
主要成果:
- 11.3%的sUCTD患者在平均11年后演变为已定义的CTD.
- 27.3%的患者在最后一次诊断时经历了器官损伤 (SLICC/DI得分平均值为1.19±0.46).
- 诊断时的年龄/症状影响了早期的损伤;抗疟疾药物和免疫抑制剂的使用与以后的损伤有关. 没有损伤的10年概率为sUCTD的62%,而SLE的23%.
结论:
- 稳定的UCTD患者可以在长期内积累器官损伤.
- 一个子集的sUCTD患者可能会演变为定义的结缔组织疾病.
- 虽然结果通常比SLE更好,但在sUCTD中长期监测损伤至关重要.
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