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原发性胆汁性胆管炎与CREST综合征:一种罕见的重叠表现及文献综述

Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature.

作者信息

Khan Shayan Iqbal, Bachani Parkash, Saleem Amna, Jameel Arifa, Khawaja Uzzam Ahmed

机构信息

Medicine, Liaquat University of Medical and Health Sciences, Hyderabad, PAK.

Medicine, Jinnah Medical and Dental College, Karachi, PAK.

出版信息

Cureus. 2020 Dec 8;12(12):e11986. doi: 10.7759/cureus.11986.

Abstract

CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder often manifesting as a consequence of superimposed autoimmune hepatitis. Herein, we present a case of a 40-year-old female with a past one-year history of hepatitis C presenting with the chief complaints of progressive thickness and tightness of the skin of hands and face and dysphagia for the past three months, along with arthralgia of the hands for the past two months, suggestive of CREST syndrome. Through this case, we intend to emphasize the association between extrahepatic manifestations and the emergence of autoantibodies in patients with hepatitis C virus (HCV) infection and discuss the clinical relevance of the autoantibodies in extrahepatic disorders, in our case, CREST syndrome. It is well-known that chronic HCV infection plays a significant part in the production of non-organ-specific autoantibodies, including antinuclear antibodies (ANA) and smooth muscle antibodies, and organ-specific autoantibodies. Clinicians must be aware of the possibility of such liver damage in patients with systemic sclerosis.

摘要

CREST(钙质沉着、雷诺现象、食管动力障碍、指端硬化和毛细血管扩张)综合征,也称为系统性硬化症的局限性皮肤型(lcSSc),是一种多系统结缔组织疾病,常因叠加自身免疫性肝炎而出现。在此,我们报告一例40岁女性患者,有丙型肝炎病史1年,主要表现为过去3个月手部和面部皮肤逐渐增厚、紧绷,吞咽困难,以及过去2个月手部关节痛,提示CREST综合征。通过这个病例,我们旨在强调丙型肝炎病毒(HCV)感染患者肝外表现与自身抗体出现之间的关联,并讨论自身抗体在肝外疾病(在我们的病例中为CREST综合征)中的临床相关性。众所周知,慢性HCV感染在包括抗核抗体(ANA)和平滑肌抗体在内的非器官特异性自身抗体以及器官特异性自身抗体的产生中起重要作用。临床医生必须意识到系统性硬化症患者存在这种肝损伤的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf28/7788041/974027e92477/cureus-0012-00000011986-i01.jpg

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