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inflammation of the bile ducts

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/03/18 22:07:21」(JST)

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Primary sclerosing cholangitis (PSC) is a disease of the bile ducts that causes inflammation and subsequent obstruction of bile ducts both at a intrahepatic (inside the liver) and extrahepatic (outside the liver) level. The inflammation impedes the flow of bile to the gut, which can ultimately lead to cirrhosis of the liver, liver failure and liver cancer. The underlying cause of the inflammation is believed to be autoimmunity;^[1] and more than 80% of those with PSC have ulcerative colitis.^[2] The definitive treatment is liver transplantation.

Signs and symptoms

PSC is characterized by recurrent episodes of cholangitis (infection of the bile ducts), with progressive biliary scarring and obstruction.

Chronic fatigue (a non-specific symptom often present in liver disease)
Severe jaundice with intense itching
Malabsorption (especially of fat) and steatorrhea (fatty stool) due to biliary obstruction, leading to decreased levels of the fat-soluble vitamins, A, D, E and K.
Signs of cirrhosis
Hepatomegaly (enlarged liver)
Portal hypertension
Ascending cholangitis, or infection of the bile duct.
Dark urine due to excess conjugated bilirubin, which is water soluble, being excreted by the kidneys
Hepatic encephalopathy (confusion caused by liver dysfunction)

Diagnosis

The diagnosis is by imaging of the bile duct, usually in the setting of endoscopic retrograde cholangiopancreatography (ERCP, endoscopy of the bile duct and pancreas), which shows "beading" (both strictures and dilation) of the intrahepatic and extrahepatic bile ducts. Another option is magnetic resonance cholangiopancreatography (MRCP), where magnetic resonance imaging is used to visualise the biliary tract.

Most patients with PSC have evidence of autoantibodies. Approximately 80% of patients have perinuclear anti-neutrophil cytoplasmic antibodies, also called p-ANCA; however, this finding is not specific for PSC. Antinuclear antibodies and anti-smooth muscle antibody are found in 20%-50% of PSC patients and, likewise, are not specific for the disease.^[1]

Other tests often done are a full blood count, liver enzymes, bilirubin levels (usually grossly elevated), renal function, electrolytes. Fecal fat determination is occasionally ordered when the symptoms of malabsorption are prominent.

The differential diagnosis can include primary biliary cirrhosis, drug induced cholestasis, cholangiocarcinoma, and HIV-associated cholangiopathy.

Etiology

The cause of PSC is unknown, although it is thought to be an autoimmune disorder. There is an increased prevalence of HLA alleles A1, B8, and DR3 in primary sclerosing cholangitis.^[1]

Pathophysiology

Inflammation damages bile ducts both inside and outside of the liver. The resulting scarring of the bile ducts blocks the flow of bile, causing cholestasis. Bile stasis and back-pressure induces proliferation of epithelial cells and focal destruction of the liver parenchyma, forming bile lakes. Chronic biliary obstruction causes portal tract fibrosis and ultimately biliary cirrhosis and liver failure.^[3]
Bile assists in the enteric breakdown and absorption of fat; the absence of bile leads to fat malabsorption and deficiencies of fat-soluble vitamins (A, D, E, K).

Epidemiology

There is a 2:1 male-to-female predilection of primary sclerosing cholangitis.^[3] The disease normally starts from age 20 to 30, though may begin in childhood. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed. There is relatively little data on the prevalence and incidence of primary sclerosing cholangitis, with studies in different countries showing annual incidence of 0.068–1.3 per 100,000 people and prevalence 0.22–8.5 per 100,000; given that PSC is closely linked with ulcerative colitis, it is likely that the risk is higher in populations where UC is more common.^[4]

Related diseases

Primary sclerosing cholangitis is associated with cholangiocarcinoma, a cancer of the biliary tree, and the lifetime risk for PSC sufferers is 10-15%. Screening for cholangiocarcinoma in patients with primary sclerosing cholangitis is encouraged, but there is no general consensus on the modality and interval of choice.

Colon cancer is also associated with PSC.

PSC has a significant association with ulcerative colitis, an inflammatory bowel disease primarily affecting the large intestine. As many as 5% of patients with ulcerative colitis may progress to develop primary sclerosing cholangitis ^[5] and approximately 70% of people with primary sclerosing cholangitis have ulcerative colitis.^[3]

Therapy

Standard treatment includes ursodiol, a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not improved liver- or overall survival.^[6] Treatment also includes medication to relieve itching (antipruritics), bile acid sequestrants (cholestyramine), antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamin A, vitamin D, vitamin E and vitamin K.

In some cases, ERCP, which may involve stenting of the common bile duct, may be necessary in order to open major blockages (dominant strictures).

Liver transplantation is the only proven long-term treatment of PSC. Indications for transplantation include recurrent bacterial cholangitis, jaundice refractory to medical and endoscopic treatment, decompensated cirrhosis and complications of portal hypertension. In one series, 1, 2, and 5 year survival following liver transplantation for PSC was 90%, 86% and 85% respectively.^[1]

Prognosis

A German study in 2007 estimated the average survival time from time of diagnosis to be approximately 25 years, and the median time until either death or liver transplantation to be approximately 10 years.^[7]

References

^ ^a ^b ^c ^d Charatcharoenwitthaya P, Lindor KD (Feb 2006). "Primary sclerosing cholangitis: diagnosis and management". Current Gastroenterology Reports 8 (1): 75–82. doi:10.1007/s11894-006-0067-8. PMID 16510038.
^ Sleisenger, MH (2006). Sleisenger and Fordtran's gastrointestinal and liver disease: pathophysiology, diagnosis, management (8th Edition ed.). Philadelphia: Saunders.
^ ^a ^b ^c Robbins SL, Kumar V, Cotran RS (2003). "Chapter 16". Robbins basic pathology (7th ed.). Philadelphia: Saunders. pp. 620–1. ISBN 0-7216-9274-5.
^ Feld JJ, Heathcote EJ (October 2003). "Epidemiology of autoimmune liver disease". J. Gastroenterol. Hepatol. 18 (10): 1118–28. doi:10.1046/j.1440-1746.2003.03165.x. PMID 12974897. http://onlinelibrary.wiley.com/doi/10.1046/j.1440-1746.2003.03165.x/full.
^ Olsson R, Danielsson A, Järnerot G, et al. (1991). "Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis". Gastroenterology 100 (5 Pt 1): 1319–23. PMID 2013375.
^ Lindor KD, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, Harnois D, Jorgensen R, Petz J, Keach J, Mooney J, Sargeant C, Braaten J, Bernard T, King D, Miceli E, Schmoll J, Hoskin T, Thapa P, Enders F (Sep 2009). "High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis.". Hepatology 50 (3): 671–3. doi:10.1002/hep.23082. PMC 2758780. PMID 19585548. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2758780/.
^ Tischendorf, J.; Hecker, H.; Krüger, M.; Manns, M.; Meier, P. (2007). "Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study". The American journal of gastroenterology 102 (1): 107–114. doi:10.1111/j.1572-0241.2006.00872.x. PMID 17037993. edit [1]. Giving survival time: 306 months

External links

PSC Partners: Patient organization with additional information
PSC Support : UK based Patient organization with additional information and discussion forum
Additional Literature about PSC
Information from The Morgan Foundation for the Study of PSC

UpToDate Contents

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1. 成人における原発性硬化性胆管炎：臨床症状および診断 primary sclerosing cholangitis in adults clinical manifestations and diagnosis
2. 成人における原発性硬化性胆管炎：マネージメント primary sclerosing cholangitis in adults management
3. 原発性硬化性胆管炎：疫学および病因 primary sclerosing cholangitis epidemiology and pathogenesis
4. 大腸癌および原発性硬化性胆管炎 colorectal cancer and primary sclerosing cholangitis
5. 小児における胆道疾患のERCP ercp for biliary disease in children

English Journal

Elevated immunoglobulin G4 level is associated with reduced colectomy-free survival in patients with primary sclerosing cholangitis and ulcerative colitis.

Navaneethan U, Gk Venkatesh P, Choudhary M, Shen B, Kiran RP.SourceDepartment of Gastroenterology, Digestive disease Institute, The Cleveland Clinic, Cleveland, OH, USA. Electronic address: udhaykumar81@gmail.com.
Journal of Crohn's & colitis.J Crohns Colitis.2013 Mar 1;7(2):e35-41. doi: 10.1016/j.crohns.2012.04.006. Epub 2012 May 1.
BACKGROUND AND AIM: Patients with primary sclerosing cholangitis (PSC) and elevated immunoglobulin (Ig) G4 have been shown to have more severe disease with a shorter time to orthotopic liver transplantation (OLT). The aim of the study was to investigate the clinical outcomes of PSC and UC in patient
PMID 22554774

Dysplasia and colorectal cancer in a patient with ulcerative colitis and primary sclerosing cholangitis: A case report and a short review of the literature.

Chaparro M, Trapero-Marugán M, Guijarro M, López C, Moreno-Otero R, Gisbert JP.SourceDepartment of Gastroenterology, Hospital Universitario de La Princesa, Instituto de Investigación Sanitaria Princesa (IP) and Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBEREHD), Madrid, Spain. Electronic address: mariachs2005@gmail.com.
Journal of Crohn's & colitis.J Crohns Colitis.2013 Mar 1;7(2):e61-5. doi: 10.1016/j.crohns.2012.04.005. Epub 2012 Apr 30.
Primary sclerosing cholangitis is a chronic progressive disorder which involves the medium size and large ducts in the intrahepatic and extrahepatic biliary tree. The great majority of cases have underlying inflammatory bowel disease, mainly ulcerative colitis. A higher risk of colorectal cancer has
PMID 22552273

Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis.

Al Mamari S, Djordjevic J, Halliday JS, Chapman RW.SourceTransitional Gastroenterology Unit, Oxford University Hospitals, Oxford, United Kingdom. Electronic address: saidalmammari@hotmail.com.
Journal of hepatology.J Hepatol.2013 Feb;58(2):329-34. doi: 10.1016/j.jhep.2012.10.013. Epub 2012 Oct 22.
BACKGROUND & AIMS: Normalization of serum alkaline phosphatase (SAP) was recently shown to correlate with better prognosis in Primary Sclerosing Cholangitis (PSC). We aimed at evaluating the impact of SAP improvement to below 1.5 the upper limit of normal (ULN) on the prognosis of this chole
PMID 23085647

Japanese Journal

自己免疫性膵炎に合併したIgG4関連硬化性胆管炎の1例 (特集第67回広島医学会総会) -- (受賞論文の要旨)

小川裕太郎,栗原啓介,佐々木民人 [他]
広島医学 68(2), 91-96, 2015-02
NAID 40020386824

Clinical profiles of patients with primary sclerosing cholangitis in the elderly

Tanaka Atsushi,Tazuma Susumu,Okazaki Kazuichi [他]
Journal of hepato-biliary-pancreatic sciences : official journal of the Japanese Society of Hepato-Biliary-Pancreatic Surgery, the Asian-Pacific Hepato-Pancreato-Biliary Association, the Japan Biliary Association 22(3), 230-236, 2015
NAID 40020403869

硬化性胆管炎の診断と治療―新診断基準の整合性―

大原弘隆,中沢貴宏,林香月,内藤格,宮部勝之,城卓志
胆道 29(2), 198-205, 2015
近年の画像診断の進歩にも関わらず，硬化性胆管炎の診断には，しばしば難渋することがある．特に，IgG4-SCはステロイド治療が奏効する比較的予後良好な疾患とされているが，その胆管像からPSCや胆管癌などの予後不良な疾患として扱われてきた症例も少なくない．そのため2012年に厚生労働省研究班と日本胆道学会との合同でIgG4関連硬化性胆管炎臨床診断基準2012が作成された．硬化性胆管炎の診断には臨床経過 …
NAID 130005077999

Related Pictures

★リンクテーブル★

リンク元	「硬化性胆管炎」
拡張検索	「primary sclerosing cholangitis」
関連記事	「sclerosing」

「硬化性胆管炎」

Primary sclerosing cholangitis
	Classification and external resources
	; Cholangiogram of primary sclerosing cholangitis.
ICD-10	K83.0
ICD-9	576.1
DiseasesDB	10643
MedlinePlus	000285
eMedicine	med/3556
MeSH	D015209

　　[★]

英: sclerosing cholangitis
関: 原発性硬化性胆管炎、胆管炎

参考

1. 硬化性胆管炎の病理

http://dspace.lib.kanazawa-u.ac.jp/dspace/bitstream/2297/24735/1/ME-PR-NAKANUMA-Y-658.pdf

「primary sclerosing cholangitis」

　　[★] 原発性硬化性胆管炎 PSC

「sclerosing」

　　[★]

adj.

硬化性の

関: cirrhotic、sclerogenous、sclerotic

[Charatchar-1] Charatcharoenwitthaya P, Lindor KD (Feb 2006). "Primary sclerosing cholangitis: diagnosis and management". Current Gastroenterology Reports 8 (1): 75–82. doi:10.1007/s11894-006-0067-8. PMID 16510038.

[2] Sleisenger, MH (2006). Sleisenger and Fordtran's gastrointestinal and liver disease: pathophysiology, diagnosis, management (8th Edition ed.). Philadelphia: Saunders.

[Robbins-3] Robbins SL, Kumar V, Cotran RS (2003). "Chapter 16". Robbins basic pathology (7th ed.). Philadelphia: Saunders. pp. 620–1. ISBN 0-7216-9274-5.

[4] Feld JJ, Heathcote EJ (October 2003). "Epidemiology of autoimmune liver disease". J. Gastroenterol. Hepatol. 18 (10): 1118–28. doi:10.1046/j.1440-1746.2003.03165.x. PMID 12974897. http://onlinelibrary.wiley.com/doi/10.1046/j.1440-1746.2003.03165.x/full.

[5] Olsson R, Danielsson A, Järnerot G, et al. (1991). "Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis". Gastroenterology 100 (5 Pt 1): 1319–23. PMID 2013375.

[Lindor-6] Lindor KD, Kowdley KV, Luketic VA, Harrison ME, McCashland T, Befeler AS, Harnois D, Jorgensen R, Petz J, Keach J, Mooney J, Sargeant C, Braaten J, Bernard T, King D, Miceli E, Schmoll J, Hoskin T, Thapa P, Enders F (Sep 2009). "High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis.". Hepatology 50 (3): 671–3. doi:10.1002/hep.23082. PMC 2758780. PMID 19585548. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2758780/.

[7] Tischendorf, J.; Hecker, H.; Krüger, M.; Manns, M.; Meier, P. (2007). "Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study". The American journal of gastroenterology 102 (1): 107–114. doi:10.1111/j.1572-0241.2006.00872.x. PMID 17037993. edit [1]. Giving survival time: 306 months

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案内

案内

sclerosing cholangitis