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硬化性肠系膜炎。

Sclerosing Mesenteritis.

机构信息

Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.

出版信息

Am J Gastroenterol. 2019 Jun;114(6):867-873. doi: 10.14309/ajg.0000000000000167.

Abstract

Sclerosing mesenteritis is a rare non-neoplastic disorder characterized by fat necrosis, chronic inflammation, and fibrosis typically of the small bowel mesentery. Our understanding of this disorder is limited by its rarity as well as inconsistent terminology used across the literature. While prior abdominal surgery or trauma, autoimmunity, infection, ischemia, and malignancy have been suggested to be involved in the pathogenesis of the disorder, it remains poorly understood. The clinical course of sclerosing mesenteritis is generally benign with a large proportion of patients diagnosed incidentally on imaging obtained for other indications. In a subset of patients, symptoms may arise from a mass effect on the bowel, lymphatics, or vasculature resulting in bowel obstruction, chylous ascites, or mesenteric ischemia. Symptomatic patients should be treated with a combination of corticosteroid and tamoxifen as first-line therapy based on retrospective case series and experience in other fibrosing disorders. Surgical intervention may be required in those with persistent obstruction despite conservative treatment, though complete resection of the mass is often not feasible given intimate involvement with the mesenteric vasculature. A careful use of terminology and communication between the radiologist, pathologist, and clinicians in the care of these patients will be essential to future efforts at understanding this disease.

摘要

硬化性肠系膜炎是一种罕见的非肿瘤性疾病,其特征为脂肪坏死、慢性炎症和纤维化,通常累及小肠系膜。由于其罕见性以及文献中使用的术语不一致,我们对这种疾病的认识受到限制。尽管先前的腹部手术或创伤、自身免疫、感染、缺血和恶性肿瘤已被认为与该疾病的发病机制有关,但目前仍知之甚少。硬化性肠系膜炎的临床病程通常是良性的,很大一部分患者是在因其他原因进行影像学检查时偶然诊断出来的。在一部分患者中,由于肿块对肠、淋巴管或脉管系统的压迫,可能会出现症状,导致肠梗阻、乳糜性腹水或肠系膜缺血。根据回顾性病例系列研究和其他纤维形成性疾病的经验,基于一线治疗的皮质类固醇和他莫昔芬联合治疗应作为有症状患者的治疗方法。对于那些尽管进行了保守治疗仍持续存在梗阻的患者,可能需要手术干预,但由于肿块与肠系膜血管密切相关,通常无法完全切除肿块。在这些患者的治疗中,放射科医生、病理学家和临床医生之间仔细使用术语和进行沟通,对于未来理解这种疾病至关重要。

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