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在轨道伪瘤之前的ANCA相关脏疾病
Ileisa Oleson1, Adeline Fecker1, Kelsey Richardson2
1School of Medicine, Oregon Health & Science University, Portland, OR, USA.
Pediatric nephrology (Berlin, Germany)
|October 19, 2023
概括
反中性细胞质抗体 (ANCA) 相关的血管炎 (AAV) 和IgG4相关的疾病 (IgG4-RD) 也可以出现类似的情况. 具有IgG4-RD特征的患者的ANCA阳性强烈表明AAV是更有可能的诊断.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
背景情况:
- 反中性细胞质抗体 (ANCA) 相关的血管炎 (AAV) 和IgG4相关的疾病 (IgG4-RD) 是不同的免疫疾病,具有重叠的特征.
- 2019年ACR/EULAR分类标准将IgG4-RD排除在外,如果ANCA是阳性的,但这并不普遍适用.
- AAV可以呈现出炎症质量和增加的IgG4+血细胞,模仿IgG4-RD.
研究的目的:
- 调查ANCA阳性的诊断影响,在IgG4相关疾病的特征呈现的患者.
- 在临床和病理发现重叠的情况下,区分AAV和IgG4-RD.
主要方法:
- 一个5岁的女性病例报告,有轨道IgG4-RD病史,呈现急性损伤.
- 对脏活检结果的分析,包括乳腺免疫半月状球炎,急性管间炎,增加的IgG4+血细胞和管状底膜 (TBM) 沉积物.
- 评估骨髓氧化酶ANCA状态.
主要成果:
- 该患者出现了急性损伤,并且具有阳性髓氧化酶ANCA.
- 脏活检揭示了乳腺免疫新月状球膜炎和急性管管间膜炎,IgG4+血细胞增加和TBM沉积.
- 虽然TBM沉积物和增加的IgG4+血细胞可以表明IgG4-RD,但ANCA的存在和PAU-Immune球体膜炎有利于AAV.
结论:
- 孤立的TBM沉积物和增加的IgG4+血细胞表明IgG4-RD.
- 在ANCA阳性和PAUCI-免疫新月状球炎的背景下,增加IgG4+血细胞更可能是由于AAV.
- 在患有IgG4-RD特征的患者中,ANCA阳性指向AAV作为更有可能的诊断.
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