宾尼尔综合征:一个未知的GCA模仿者
Arifa Javed1, Sadia Arooj Javed2, Barbara Ostrov1
1Albany Medical Center Department of Rheumatology, Albany, USA.
Case reports in rheumatology
|August 20, 2024
概括
巨细胞动脉炎 (GCA) 可能是宾尼尔综合征 (BNS) 的罕见表现,这是沃尔登斯特罗姆巨球蛋白血症的并发症. 这一案例凸显了在GCA演讲中考虑BNS的重要性.
科学领域:
- 神经学和眼科 眼科
- 类风湿病学 类风湿病学
- 血液学 血液学 血液学
背景情况:
- 巨细胞动脉炎 (GCA) 是一种影响中大动脉的血管炎,常常呈现部症状.
- 动脉活检是GCA诊断的黄金标准,其灵敏度有限.
- 宾尼尔综合征 (Bing-Neel Syndrome,简称BNS) 是一种罕见的中枢神经系统并发症,是沃尔登斯特罗姆巨球蛋白血症 (Waldenstrom Macroglobulinemia,简称WM) 的一种罕见并发症.
研究的目的:
- 报告一个独特的Bing-Neel综合征 (BNS) 病例,呈现为经活检证明的巨细胞动脉炎 (GCA).
- 强调在诊断GCA时考虑罕见并发症的重要性.
主要方法:
- 一个77岁的女性视力丧失的案例报告.
- 诊断工作包括后视镜,动脉活检,偏蛋白质血的评估和骨髓活检.
- 开始治疗所谓的GCA和确定的WM.
主要成果:
- 动脉活检证实了GCA.
- 检查显示有类蛋白质血症,骨髓活检证实了沃尔登斯特罗姆巨型球蛋白血症 (WM).
- 患者的眼科并发症在WM治疗后稳定.
结论:
- 宾尼尔综合征 (BNS) 可以呈现为巨细胞关节炎 (GCA),尽管它很罕见.
- 综合性诊断评估对于识别潜在疾病至关重要,如怀疑GCA病例中的WM.
- 及时治疗潜在的血液性恶性瘤可以稳定眼科表现.
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