系统性硬化症由阿扎西奥普林诱导的阳性免疫缺陷相关的淋巴增殖障碍复杂化:一个病例报告
Ryota Okazaki1, Genki Inui1, Yoshihiro Funaki1
1Division of Respiratory Medicine and Rheumatology, Faculty of Medicine, Tottori University, Tottori, Japan.
Modern rheumatology case reports
|March 6, 2025
概括
本病例报告详细介绍了一例罕见的阿扎西奥普林诱导的淋巴增殖障碍,该病例发生在患有全身性硬化症的患者身上. 它强调需要对免疫抑制患者这种情况进行警监测.
科学领域:
- 免疫学 免疫学 免疫学
- 在瘤学瘤学.
- 类风湿病学 类风湿病学
背景情况:
- 淋巴增殖性疾病是在接受免疫抑制的自身免疫疾病患者中不常见的并发症.
- 系统性硬化症,特别是抗RNA聚合酶III抗体,与恶性瘤的风险增加有关.
- 与原性免疫缺陷相关的淋巴增殖性疾病 (LPDs) 是免疫抑制群体中公认的担忧.
研究的目的:
- 在患有全身性硬化症的患者中报告第一例与阿扎西奥普林相关的淋巴增殖障碍.
- 突出系统性硬化症患者接受免疫抑制疗法的LPD潜在风险,特别是那些具有抗RNA聚合酶III抗体的患者.
- 强调在这个患者群体中监测LPD的重要性.
主要方法:
- 一名74岁的男性患有扩散性皮肤系统性硬化症,抗RNA聚合酶III抗体和间歇性肺炎的病例报告.
- 患者最初接受了普雷迪尼索隆和环胺,其次是阿扎西奥普林,宁泰达尼布和马西坦坦.
- 在30个月的阿扎西奥普林 (AZA) 治疗后,下和部结节/的发展;通过皮肤活检诊断扩散性大B细胞淋巴瘤.
主要成果:
- 患者在30个月的阿扎西奥普林治疗后发展出扩散性大B细胞淋巴瘤.
- 停止阿扎西奥普林治疗导致患者的的消失.
- 在停止使用阿扎西奥普林后,没有进一步的病变.
结论:
- 这一病例代表了系统性硬化症中阿扎西奥普林相关的淋巴增殖性疾病的第一个记录实例.
- 系统性硬化症患者,特别是具有抗RNA聚合酶III抗体的患者,在免疫抑制疗法期间需要密切监测淋巴增殖性疾病.
- 对包括LPD在内的恶性瘤的警监测对于接受免疫抑制剂的全身性硬化症患者至关重要.
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