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一项对梅-罗综合征患者的 30 年随访研究显示,该病与炎症性肠病有关。

A 30-year follow-up study of patients with Melkersson-Rosenthal syndrome shows an association to inflammatory bowel disease.

机构信息

a Department of Otorhinolaryngology, Head and Neck Surgery , Helsinki University Hospital and University of Helsinki , Helsinki , Finland.

b Department of Pediatrics , Helsinki University Hospital and University of Helsinki , Helsinki , Finland.

出版信息

Ann Med. 2019 Mar;51(2):149-155. doi: 10.1080/07853890.2019.1591634. Epub 2019 Apr 13.

Abstract

Melkersson-Rosenthal syndrome (MRS) is often classified under the term orofacial granulomatosis (OFG). A part of OFG patients eventually develop Crohn's disease (CD), but the relationship between MRS and CD is unknown. To evaluate the long-term outcomes of MRS patients, with specific interest in bowel-related symptoms. This follow-up study consisted of adult patients with MRS - including the monosymptomatic form, cheilitis granulomatosa (CG) - who had participated in our earlier MRS study (diagnosed after 1995 in Helsinki University Hospital). A phone interview was conducted with 27 patients (77.1% of the patients from the earlier study) and included questions about orofacial symptoms, facial palsy, intestinal symptoms, concomitant illnesses, medications, possible food avoidances and family history. Stool samples were collected to measure faecal calprotectin, a surrogate marker for intestinal inflammation. The median follow-up time from symptom onset was 30 years. Three (11.1%) patients had developed inflammatory bowel disease (IBD); one CG patient developed CD, and two MRS patients with facial palsy developed ulcerative colitis. In addition, several other patients reported intestinal symptoms, but the examination of faecal calprotectin did not indicate intestinal inflammation. There was a connection between MRS and IBD - not only CD but ulcerative colitis as well. Key message Melkersson-Rosenthal syndrome (MRS) is a chronic condition often classified as a subtype of orofacial granulomatosis. Oral manifestations are common in MRS. We found a connection between MRS and inflammatory bowel disease, not only Crohn's disease but ulcerative colitis as well.

摘要

梅尔基奥尔森-罗森塔尔综合征(MRS)通常归类于口面肉芽肿病(OFG)。一部分 OFG 患者最终会发展为克罗恩病(CD),但 MRS 和 CD 之间的关系尚不清楚。为了评估 MRS 患者的长期预后,特别是关注与肠道相关的症状。这项随访研究包括曾参加过我们早期 MRS 研究的成年 MRS 患者(1995 年后在赫尔辛基大学医院诊断),包括单症状型和肉芽肿性唇炎(CG)。对 27 名患者(早期研究患者的 77.1%)进行了电话访谈,问题包括口面症状、面瘫、肠道症状、合并症、药物治疗、可能的食物禁忌和家族史。收集粪便样本以测量粪便钙卫蛋白,这是肠道炎症的替代标志物。从症状发作到中位随访时间为 30 年。3 名(11.1%)患者发展为炎症性肠病(IBD);1 名 CG 患者发展为 CD,2 名面神经麻痹的 MRS 患者发展为溃疡性结肠炎。此外,还有其他几名患者报告有肠道症状,但粪便钙卫蛋白检查并未显示肠道炎症。MRS 与 IBD 之间存在关联,不仅是 CD,还有溃疡性结肠炎。关键信息梅尔基奥尔森-罗森塔尔综合征(MRS)是一种慢性疾病,通常归类为口面肉芽肿病的一个亚型。口腔表现常见于 MRS。我们发现 MRS 与炎症性肠病之间存在关联,不仅与 CD 有关,还与溃疡性结肠炎有关。

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本文引用的文献

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J Pediatr Gastroenterol Nutr. 2017 Oct;65(4):388-393. doi: 10.1097/MPG.0000000000001554.
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Orofacial granulomatosis.口面部肉芽肿病
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Orofacial granulomatosis--a 20-year review.口面部肉芽肿病——20年回顾
Oral Dis. 2009 Jan;15(1):46-51. doi: 10.1111/j.1601-0825.2008.01500.x.
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Orofacial granulomatosis: review on aetiology and pathogenesis.口面部肉芽肿病:病因及发病机制综述
J Oral Pathol Med. 2008 Apr;37(4):191-5. doi: 10.1111/j.1600-0714.2007.00591.x.
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Melkersson-Rosenthal syndrome.梅尔克森-罗森塔尔综合征
Otolaryngol Head Neck Surg. 2008 Feb;138(2):246-51. doi: 10.1016/j.otohns.2007.11.015.

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