这是什么联系? Sarcoidosis 和 综合变性免疫缺陷: 一个单一中心的回顾性病例系列
Sarah MacDowell1, Arindam Singha1, Elliott Crouser1
1Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, College of Medicine, The Ohio State University Wexner Medical Center, Columbus, OH.
从组合变异性免疫缺陷 (CVID) 区分沙尔科病是具有挑战性的. 这项研究确定了患者的关键特征,如复发性感染和特定的CT发现,以指导在肉病患者的免疫球蛋白测试.
科学领域:
- 免疫学 免疫学 免疫学
- 肺部病理学 肺部病理学
- 类风湿病学 类风湿病学
背景情况:
- 沙尔科毒症呈现出颗粒瘤,通常会影响肺部,眼睛,中枢神经系统和皮肤.
- 综合变性免疫缺陷症 (CVID) 涉及低血和复发性感染.
- 存在重叠,因为CVID可以表现为颗粒瘤 (例如GLILD),使诊断复杂化.
研究的目的:
- 为了澄清sarcoidosis和CVID之间的关系.
- 制定关于在类病患者中进行定量免疫球蛋白检测的指导方针.
- 为了确定特定的临床特征,保证CVID查在肉类发病.
主要方法:
- 在中西部学术中心对17年数据的回顾性审查.
- 利用ICD-9和ICD-10的代码来识别沙尔科毒症和CVID病例.
- 分析了1920例肉病症病例和231例CVID病例.
主要成果:
- 鉴定了8名患者 (0.042%) 患有CVID和肉类突症的诊断.
- 发现在沙尔科毒症中进行全方位免疫球蛋白查是昂贵的,并且可以识别其他导致低血的原因.
- 确定特定的患者特征,表明需要免疫球蛋白检测.
结论:
- 避免在所有肉病患者中进行普遍的定量免疫球蛋白查.
- 专注于对有复发性鼻肺感染病史的患者进行测试.
- 考虑对个人/家庭自身免疫病史,CT发现 (非伸缩支气管) 或特定的肺叶参与模式的个人进行测试.
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