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

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 hepatit...

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Autores principales: Khan, Shayan Iqbal, Bachani, Parkash, Saleem, Amna, Jameel, Arifa, Khawaja, Uzzam Ahmed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7788041/
https://www.ncbi.nlm.nih.gov/pubmed/33425554
http://dx.doi.org/10.7759/cureus.11986
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author Khan, Shayan Iqbal
Bachani, Parkash
Saleem, Amna
Jameel, Arifa
Khawaja, Uzzam Ahmed
author_facet Khan, Shayan Iqbal
Bachani, Parkash
Saleem, Amna
Jameel, Arifa
Khawaja, Uzzam Ahmed
author_sort Khan, Shayan Iqbal
collection PubMed
description 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.
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spelling pubmed-77880412021-01-08 Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature Khan, Shayan Iqbal Bachani, Parkash Saleem, Amna Jameel, Arifa Khawaja, Uzzam Ahmed Cureus Dermatology 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. Cureus 2020-12-08 /pmc/articles/PMC7788041/ /pubmed/33425554 http://dx.doi.org/10.7759/cureus.11986 Text en Copyright © 2020, Khan et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Dermatology
Khan, Shayan Iqbal
Bachani, Parkash
Saleem, Amna
Jameel, Arifa
Khawaja, Uzzam Ahmed
Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature
title Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature
title_full Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature
title_fullStr Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature
title_full_unstemmed Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature
title_short Primary Biliary Cholangitis and CREST Syndrome: A Rare, Overlapping Presentation With a Review of the Literature
title_sort primary biliary cholangitis and crest syndrome: a rare, overlapping presentation with a review of the literature
topic Dermatology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7788041/
https://www.ncbi.nlm.nih.gov/pubmed/33425554
http://dx.doi.org/10.7759/cureus.11986
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