Cargando…

Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but...

Descripción completa

Detalles Bibliográficos
Autores principales: Koksal, Deniz, Koksal, Aydin Seref, Gurakar, Ahmet
Formato: Online Artículo Texto
Lenguaje:English
Publicado: XIA & HE Publishing Inc. 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5075009/
https://www.ncbi.nlm.nih.gov/pubmed/27777894
http://dx.doi.org/10.14218/JCTH.2016.00024
_version_ 1782461795096592384
author Koksal, Deniz
Koksal, Aydin Seref
Gurakar, Ahmet
author_facet Koksal, Deniz
Koksal, Aydin Seref
Gurakar, Ahmet
author_sort Koksal, Deniz
collection PubMed
description Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC. The pulmonary manifestations of PBC include abnormalities in gas transfer and pulmonary function, subclinical alveolitis, interstitial lung disease, granulomatous lung disease, airway disease, pulmonary hypertension, pulmonary hemorrhage and pleural effusion.
format Online
Article
Text
id pubmed-5075009
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher XIA & HE Publishing Inc.
record_format MEDLINE/PubMed
spelling pubmed-50750092016-10-24 Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis Koksal, Deniz Koksal, Aydin Seref Gurakar, Ahmet J Clin Transl Hepatol Review Article Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic liver disease caused by diffuse inflammation, destruction and fibrosis of the intrahepatic bile ducts, ultimately leading to cirrhosis, portal hypertension and liver failure. The pathogenesis of PBC is incompletely understood, but current data suggest roles for genetic susceptibility and environmental factors. PBC is often thought of as an organ-specific autoimmune disease, which mainly targets the liver; however, lung tissue is also a site for autoimmune involvement of PBC. The pulmonary manifestations of PBC include abnormalities in gas transfer and pulmonary function, subclinical alveolitis, interstitial lung disease, granulomatous lung disease, airway disease, pulmonary hypertension, pulmonary hemorrhage and pleural effusion. XIA & HE Publishing Inc. 2016-08-14 2016-09-28 /pmc/articles/PMC5075009/ /pubmed/27777894 http://dx.doi.org/10.14218/JCTH.2016.00024 Text en © 2016 The Second Affiliated Hospital of Chongqing Medical University. Published by XIA & HE Publishing Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial 4.0 Unported License, permitting all non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Koksal, Deniz
Koksal, Aydin Seref
Gurakar, Ahmet
Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis
title Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis
title_full Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis
title_fullStr Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis
title_full_unstemmed Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis
title_short Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis
title_sort pulmonary manifestations among patients with primary biliary cirrhosis
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5075009/
https://www.ncbi.nlm.nih.gov/pubmed/27777894
http://dx.doi.org/10.14218/JCTH.2016.00024
work_keys_str_mv AT koksaldeniz pulmonarymanifestationsamongpatientswithprimarybiliarycirrhosis
AT koksalaydinseref pulmonarymanifestationsamongpatientswithprimarybiliarycirrhosis
AT gurakarahmet pulmonarymanifestationsamongpatientswithprimarybiliarycirrhosis