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肉芽肿伴多血管炎伴血栓性血管炎和坏死性脑脊髓膜炎及脑瘤样病变。

Granulomatosis with Polyangiitis Presenting with Thrombovasculitic and Necrotizing Pachy- and Leptomeningitis Accompanied by a Brain Tumor-like Lesion.

机构信息

Department of Neurology, National Hospital Organization Beppu Medical Center, Japan.

Department of Pathology, National Hospital Organization Beppu Medical Center, Japan.

出版信息

Intern Med. 2023 Nov 1;62(21):3241-3246. doi: 10.2169/internalmedicine.0833-22. Epub 2023 Mar 22.

DOI:10.2169/internalmedicine.0833-22
PMID:36948617
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10686742/
Abstract

We report a case of granulomatosis with polyangiitis (GPA) presenting with hypertrophic pachymeningitis with a huge brain tumor-like lesion. A 57-year-old man acutely developed consciousness disturbance. Magnetic resonance imaging revealed a right frontal lobe mass with thickened, contrast-enhanced dura. Computed tomography revealed sinusitis and multiple lung nodules. The presence of proteinase 3-anti-neutrophil cytoplasmic antibody indicated GPA. Histopathology of the excised brain tissues revealed thrombovasculitis with heavy neutrophilic infiltration in the pachy- and leptomeninges covering an ischemic cerebral cortex. The patient improved with corticosteroids and rituximab. Our case warrants considering GPA as a cause of hypertrophic pachymeningitis with brain-tumor like lesions.

摘要

我们报告了一例肉芽肿伴多血管炎（GPA）病例，其表现为肥厚性硬脑膜炎伴巨大脑瘤样病变。一名 57 岁男性急性出现意识障碍。磁共振成像显示右额叶肿块伴增厚、对比增强硬脑膜。计算机断层扫描显示鼻窦炎和多个肺结节。蛋白酶 3-抗中性粒细胞胞质抗体的存在提示 GPA。切除脑组织的组织病理学显示血栓性血管炎，伴有覆盖缺血大脑皮层的厚层和软脑膜中的大量中性粒细胞浸润。患者接受皮质类固醇和利妥昔单抗治疗后病情改善。我们的病例提示应考虑 GPA 是导致肥厚性硬脑膜炎伴脑瘤样病变的原因之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/8345cdf52cdc/1349-7235-62-3241-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/c74326cef403/1349-7235-62-3241-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/6c9692e4e424/1349-7235-62-3241-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/938166b3a337/1349-7235-62-3241-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/c71caf96baf0/1349-7235-62-3241-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/814858dfd66a/1349-7235-62-3241-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/1048504788d7/1349-7235-62-3241-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/8345cdf52cdc/1349-7235-62-3241-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/c74326cef403/1349-7235-62-3241-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/6c9692e4e424/1349-7235-62-3241-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/938166b3a337/1349-7235-62-3241-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/c71caf96baf0/1349-7235-62-3241-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/814858dfd66a/1349-7235-62-3241-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/1048504788d7/1349-7235-62-3241-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e218/10686742/8345cdf52cdc/1349-7235-62-3241-g007.jpg

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本文引用的文献

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IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions.IgG4 相关肥厚性脑脊膜炎伴颅内和脑内肿瘤样病变。
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