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在IgG4相关疾病和等离子体细胞类型异常多中心卡斯特曼病之间的肺部参与的临床和放射学差异

Jiamin Zhou¹, Xueqing Liu¹, Jian Li²

¹Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, No.1 Shuaifuyuan, Dongcheng District, Beijing, 100730, China.

|January 3, 2025

概括

区分免疫球蛋白G4相关疾病 (IgG4-RD) 和血细胞类型异常多中心卡斯特曼病 (PC-iMCD) 是至关重要的. 放射学发现,如广泛的结节或囊表明PC-iMCD,而加厚的支气管束指向IgG4-RD.

科学领域:

肺部病理学肺部病理学
类风湿病学类风湿病学
病理学病理学病理学

背景情况:

免疫球蛋白G4相关疾病 (IgG4-RD) 和血细胞类型异常多中心卡斯特曼病 (PC-iMCD) 具有重叠的临床和放射性特征.
由于不同的预后和治疗策略,准确的差异诊断至关重要.
专注于肺部参与这些疾病的比较研究是有限的.

研究的目的:

阐明IgG4-RD和PC-iMCD之间肺部参与的临床和放射学区别.
为了帮助这些两个相关条件的具有挑战性的差异诊断.

主要方法:

进行了临床数据和胸部CT检测结果的比较分析.
被诊断为IgG4-RD或PC-iMCD且表现出肺部参与的患者被追溯纳入.

主要成果:

分析了178名IgG4-RD和61名肺部受影响的PC-iMCD患者.
在PC-iMCD患者中,出现了更多的体内/呼吸系统症状,贫血,血栓细胞瘤,低albuminemia,以及显著更高的CRP,ESR和IL-6水平.
在IgG4-RD中,升高的IgG4水平更加明显,而PC-iMCD显示IgG,IgA和IgM的水平更高.
在放射学上,广泛的结节性病变和囊是PC-iMCD的特征,而扩散的加厚支气管束则更能表明IgG4-RD.

结论:

PC-iMCD通常比IgG4-RD更具侵略性,表现为更明显的症状和全身炎症.
胸部CT发现是关键的区分因素:广泛的结节/囊表明PC-iMCD,而加厚的支气管血管捆绑表明IgG4-RD.
这些发现可以提高诊断的准确性,并指导适当的患者管理.

关键词:

异常多中心的卡斯特曼病异常多中心的卡斯特曼病与IgG4相关的疾病肺部参与肺部参与淋巴细胞细胞增殖性疾病.

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