Natural history and management of primary biliary cirrhosis

Nadya Al-Harthy,1 Teru Kumagi21Gastroenterology and Hepatology, Royal Hospital, Muscat, Oman; 2Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, JapanAbstract: Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cho...

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Autores principales:	Al-Harthy N, Kumagi T
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2012
Materias:	Diseases of the digestive system. Gastroenterology RC799-869
Acceso en línea:	https://doaj.org/article/67b2e279fafb4e139d88a6ee53da763a
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spelling	oai:doaj.org-article:67b2e279fafb4e139d88a6ee53da763a2021-12-02T04:21:55ZNatural history and management of primary biliary cirrhosis1179-1535https://doaj.org/article/67b2e279fafb4e139d88a6ee53da763a2012-12-01T00:00:00Zhttp://www.dovepress.com/natural-history-and-management-of-primary-biliary-cirrhosis-a11675https://doaj.org/toc/1179-1535Nadya Al-Harthy,1 Teru Kumagi21Gastroenterology and Hepatology, Royal Hospital, Muscat, Oman; 2Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, JapanAbstract: Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. It is a rare disease with prevalence of less than one in 2000. Its prevalence in developing countries is increasing presumably because of growth in recognition and knowledge of the disease. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The prognosis of PBC has improved because of early diagnosis and use of ursodeoxycholic acid, the only established medical treatment for this disorder. When administered at adequate doses of 13&ndash;15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. However, some patients do not respond adequately to ursodeoxycholic acid and might need alternative therapeutic approaches.Keywords: primary biliary cirrhosis, natural history, long-term outcome, ursodeoxycholic acid, biochemical response, target therapyAl-Harthy NKumagi TDove Medical PressarticleDiseases of the digestive system. GastroenterologyRC799-869ENHepatic Medicine: Evidence and Research, Vol 2012, Iss default, Pp 61-71 (2012)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the digestive system. Gastroenterology RC799-869
spellingShingle	Diseases of the digestive system. Gastroenterology RC799-869 Al-Harthy N Kumagi T Natural history and management of primary biliary cirrhosis
description	Nadya Al-Harthy,1 Teru Kumagi21Gastroenterology and Hepatology, Royal Hospital, Muscat, Oman; 2Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Ehime, JapanAbstract: Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. It is a rare disease with prevalence of less than one in 2000. Its prevalence in developing countries is increasing presumably because of growth in recognition and knowledge of the disease. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The prognosis of PBC has improved because of early diagnosis and use of ursodeoxycholic acid, the only established medical treatment for this disorder. When administered at adequate doses of 13&ndash;15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. However, some patients do not respond adequately to ursodeoxycholic acid and might need alternative therapeutic approaches.Keywords: primary biliary cirrhosis, natural history, long-term outcome, ursodeoxycholic acid, biochemical response, target therapy
format	article
author	Al-Harthy N Kumagi T
author_facet	Al-Harthy N Kumagi T
author_sort	Al-Harthy N
title	Natural history and management of primary biliary cirrhosis
title_short	Natural history and management of primary biliary cirrhosis
title_full	Natural history and management of primary biliary cirrhosis
title_fullStr	Natural history and management of primary biliary cirrhosis
title_full_unstemmed	Natural history and management of primary biliary cirrhosis
title_sort	natural history and management of primary biliary cirrhosis
publisher	Dove Medical Press
publishDate	2012
url	https://doaj.org/article/67b2e279fafb4e139d88a6ee53da763a
work_keys_str_mv	AT alharthyn naturalhistoryandmanagementofprimarybiliarycirrhosis AT kumagit naturalhistoryandmanagementofprimarybiliarycirrhosis
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Natural history and management of primary biliary cirrhosis

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