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以硬脑膜炎为表现的韦格纳肉芽肿病：利妥昔单抗治疗后临床及影像学缓解

Wegener's granulomatosis presenting with pachymeningitis: clinical and imaging remission by rituximab.

作者信息

Just Søren Andreas, Knudsen John Bonde, Nielsen Mie Kiszka, Junker Peter

机构信息

Department of Internal Medicine, Odense University Hospital, Valdemarsgade 53, 5700 Svendborg, Denmark.

出版信息

ISRN Rheumatol. 2011;2011:608942. doi: 10.5402/2011/608942. Epub 2011 Jun 8.

DOI:10.5402/2011/608942
PMID:22389798
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3263746/
Abstract

A 27-year-old woman was admitted for intractable right-sided neck, ear, and jaw pain with gradual development of tinnitus and hearing loss. A cerebral MRI showed meningo-dural enhancement, and additional diagnostic workup revealed a right pulmonary infiltrate and positive PR-3 ANCA. Biopsies from nasal mucosa and lung showed chronic inflammation with granuloma formation. Based on these findings the patient was diagnosed with Wegener's granulomatosis with pachymeningitis. There was no clinical response to oral Prednisolone and Cyclophosphamide, but complete clinical and imaging remission was achieved by adding Rituximab.

摘要

一名27岁女性因右侧颈部、耳部和下颌顽固性疼痛入院，耳鸣和听力损失逐渐加重。脑部MRI显示脑膜-硬脑膜强化，进一步的诊断检查发现右肺浸润及PR-3抗中性粒细胞胞浆抗体阳性。鼻黏膜和肺部活检显示慢性炎症伴肉芽肿形成。基于这些发现，患者被诊断为伴有硬脑膜炎的韦格纳肉芽肿。口服泼尼松龙和环磷酰胺无临床反应，但加用利妥昔单抗后实现了临床和影像学完全缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcb3/3263746/d579d332560e/RHEUMATOLOGY2011-608942.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcb3/3263746/111f739cdd6a/RHEUMATOLOGY2011-608942.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcb3/3263746/d579d332560e/RHEUMATOLOGY2011-608942.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcb3/3263746/111f739cdd6a/RHEUMATOLOGY2011-608942.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dcb3/3263746/d579d332560e/RHEUMATOLOGY2011-608942.002.jpg

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Clin Rheumatol. 2010 Jan;29(1):107-10. doi: 10.1007/s10067-009-1291-z. Epub 2009 Oct 3.
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High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients.变应性肉芽肿性血管炎、韦格纳肉芽肿病和显微镜下多血管炎中静脉血栓栓塞事件的高发生率，但结节性多动脉炎中无此现象：一项对1130例患者的系统回顾性研究
Ann Rheum Dis. 2009 Apr;68(4):564-7. doi: 10.1136/ard.2008.099051. Epub 2008 Nov 17.
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Curr Rheumatol Rev. 2024;20(3):270-283. doi: 10.2174/0115733971263964231020072640.
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