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干燥综合征中的肠系膜脂膜炎:一种需考虑的新的全身表现?

Mesenteric Panniculitis in Sjögren's Syndrome: A New Systemic Manifestation to Consider?

作者信息

Mendes Patrícia Afonso, Ferreira Diana Marques, Temido Helena, Pina Rui, de Carvalho Armando

机构信息

Department of Internal Medicine A, Coimbra Hospital and University Center, Coimbra, Portugal.

University Clinic of Internal Medicine, Faculty of Medicine, University of Coimbra, Coimbra, Portugal.

出版信息

Eur J Case Rep Intern Med. 2018 Apr 24;5(4):000788. doi: 10.12890/2018_000788. eCollection 2018.

Abstract

UNLABELLED

The association between mesenteric panniculitis and Sjögren's syndrome, although rare, is starting to be recognized. Usually, mesenteric panniculitis is symptomatic, presenting with either general or gastrointestinal symptoms. Sjögren's syndrome is an autoimmune disease that typically affects secretory glands, but may have serious systemic involvement. We report the case of a 77-year-old patient in whom accidental discovery of asymptomatic mesenteric panniculitis on computed tomography led to the diagnosis of Sjögren's syndrome with several systemic manifestations.

LEARNING POINTS

Mesenteric panniculitis is a rare condition, sometimes associated with autoimmune diseases, which can make the differential diagnosis with more frequent systemic diseases difficult. There is no specific treatment for mesenteric panniculitis, but it is agreed that only symptomatic patients should be treated.Although Sjögren's syndrome typically presents with xerostomia and xerophthalmia (dry eyes and mouth syndrome, DEMS), it should always be kept in mind that systemic manifestations can occur, especially in more severe cases.

摘要

未标注

肠系膜脂膜炎与干燥综合征之间的关联虽然罕见,但已开始被认识到。通常,肠系膜脂膜炎有症状,表现为全身症状或胃肠道症状。干燥综合征是一种自身免疫性疾病,通常影响分泌腺,但可能有严重的全身累及。我们报告一例77岁患者,其在计算机断层扫描时意外发现无症状性肠系膜脂膜炎,进而诊断为伴有多种全身表现的干燥综合征。

学习要点

肠系膜脂膜炎是一种罕见疾病,有时与自身免疫性疾病相关,这会使与更常见的全身性疾病的鉴别诊断变得困难。肠系膜脂膜炎没有特异性治疗方法,但一致认为仅应对有症状的患者进行治疗。虽然干燥综合征通常表现为口干和眼干(干眼和口干综合征,DEMS),但应始终牢记可能会出现全身表现,尤其是在更严重的病例中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/180c/6346754/e9800ced4030/788_Fig1.jpg

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