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诊断难题：IgG4相关性硬化性肠系膜膜炎酷似包绕肠系膜上动脉的腹部恶性肿瘤。

Diagnostic Dilemma: IgG4-Related Sclerosing Mesenteritis Mimicking an Abdominal Malignancy Enveloping the Superior Mesenteric Artery.

作者信息

Ahmad Kamran, Saleh Mahnosh, Kakakhel Musa, Yasin Hasina, Ali Yasir, Rehman Hamid Ur, Saeed Usama

机构信息

Internal Medicine, Hayatabad Medical Complex Peshawar, Peshawar, PAK.

Internal Medicine, Ayub Teaching Hospital, Abbottabad, PAK.

出版信息

Cureus. 2024 Apr 17;16(4):e58480. doi: 10.7759/cureus.58480. eCollection 2024 Apr.

DOI:10.7759/cureus.58480
PMID:38765330
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11101154/
Abstract

Sclerosing mesenteritis, a rare fibroinflammatory disease affecting the mesentery, presents a diagnostic challenge due to its varied clinical manifestations and unknown etiology. We present a case of a 50-year-old female presenting with epigastric pain and weight loss, initially suspected of abdominal malignancy. Imaging revealed a mesenteric mass, and histopathological examination confirmed dense lymphoplasmacytic infiltrate with storiform fibrosis, along with elevated serum IgG4 levels, indicative of IgG4-related sclerosing mesenteritis. Treatment with thalidomide and prednisolone resulted in significant mass regression and symptom improvement. Our case highlights the importance of considering sclerosing mesenteritis in the differential diagnosis of abdominal masses and suggests a potential therapeutic approach for this rare condition. Further research is warranted to elucidate its pathogenesis and optimize management strategies.

摘要

硬化性肠系膜脂膜炎是一种影响肠系膜的罕见纤维炎性疾病，因其临床表现多样且病因不明，给诊断带来了挑战。我们报告一例50岁女性患者，表现为上腹部疼痛和体重减轻，最初怀疑为腹部恶性肿瘤。影像学检查发现肠系膜肿块，组织病理学检查证实有密集的淋巴浆细胞浸润伴席纹状纤维化，同时血清IgG4水平升高，提示为IgG4相关性硬化性肠系膜脂膜炎。沙利度胺和泼尼松龙治疗导致肿块明显缩小，症状改善。我们的病例强调了在腹部肿块鉴别诊断中考虑硬化性肠系膜脂膜炎的重要性，并提示了针对这种罕见疾病的潜在治疗方法。有必要进一步研究以阐明其发病机制并优化管理策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/f33194f76977/cureus-0016-00000058480-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/18b74825e052/cureus-0016-00000058480-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/68fe9c6efba7/cureus-0016-00000058480-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/84f2d0a1b4a3/cureus-0016-00000058480-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/7e1383067abe/cureus-0016-00000058480-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/f33194f76977/cureus-0016-00000058480-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/18b74825e052/cureus-0016-00000058480-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/68fe9c6efba7/cureus-0016-00000058480-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/84f2d0a1b4a3/cureus-0016-00000058480-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/7e1383067abe/cureus-0016-00000058480-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/971b/11101154/f33194f76977/cureus-0016-00000058480-i05.jpg

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本文引用的文献

1
Sclerosing Mesenteritis as an Uncommon Site of Involvement of IgG4-Related Disease: A Case Report With an Updated Review of the Literature.硬化性肠系膜脂膜炎作为IgG4相关疾病少见的受累部位：1例病例报告及文献复习更新
Cureus. 2022 May 16;14(5):e25041. doi: 10.7759/cureus.25041. eCollection 2022 May.
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Sclerosing mesenteritis: a comprehensive clinical review.硬化性肠系膜脂膜炎：一项全面的临床综述
Ann Transl Med. 2018 Sep;6(17):336. doi: 10.21037/atm.2018.07.01.
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Current approach to the diagnosis of IgG4-related disease - Combination of comprehensive diagnostic and organ-specific criteria.
目前 IgG4 相关疾病的诊断方法——综合诊断和器官特异性标准的结合。
Mod Rheumatol. 2017 May;27(3):381-391. doi: 10.1080/14397595.2017.1290911.
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Mesenteric panniculitis: an update.肠系膜脂膜炎：最新进展
Expert Rev Gastroenterol Hepatol. 2015 Jan;9(1):67-78. doi: 10.1586/17474124.2014.939632. Epub 2014 Sep 14.
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IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology.IgG4 相关硬化性肠系膜炎：一种病因不明的罕见肠系膜疾病。
Pathol Int. 2012 Apr;62(4):281-6. doi: 10.1111/j.1440-1827.2012.02805.x. Epub 2012 Mar 1.
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Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011.IgG4 相关疾病（IgG4-RD）的综合诊断标准，2011 年。
Mod Rheumatol. 2012 Feb;22(1):21-30. doi: 10.1007/s10165-011-0571-z. Epub 2012 Jan 5.
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Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.硬化性肠系膜脂膜炎：92例患者的临床特征、治疗及预后
Clin Gastroenterol Hepatol. 2007 May;5(5):589-96; quiz 523-4. doi: 10.1016/j.cgh.2007.02.032.