Sclerosing Cholangitis

Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.

Also Known As:

Networked: 500 relevant articles (8 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pancreatitis
2.	Cholangiocarcinoma
3.	Biliary Liver Cirrhosis (Primary Biliary Cirrhosis)
4.	Cholestasis
5.	Colorectal Neoplasms (Colorectal Cancer)

Experts

1.	Nakanuma, Yasuni: 14 articles (06/2014 - 10/2007)
2.	Trauner, Michael: 12 articles (04/2013 - 05/2004)
3.	Zen, Yoh: 11 articles (10/2014 - 10/2007)
4.	Fickert, Peter: 11 articles (07/2014 - 07/2004)
5.	Okazaki, Kazuichi: 10 articles (10/2015 - 12/2005)
6.	Nakazawa, Takahiro: 10 articles (06/2015 - 12/2004)
7.	Kamisawa, Terumi: 9 articles (09/2015 - 12/2008)
8.	Ohara, Hirotaka: 9 articles (06/2015 - 12/2004)
9.	Vergani, Diego: 8 articles (09/2015 - 06/2008)
10.	Mieli-Vergani, Giorgina: 8 articles (09/2015 - 04/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sclerosing Cholangitis:

1.	Ursodeoxycholic Acid (Urso)FDA LinkGeneric 06/01/2002 - "(3) Likewise, ursodeoxycholic acid seems to have a biochemical effect but no clear clinical effect in sclerosing cholangitis or in treating the hepatic complications of cystic fibrosis. " 05/01/1992 - "The administration of ursodeoxycholic acid (UDCA) has been reported to improve cholestasis in patients with primary biliary cirrhosis or sclerosing cholangitis. " 02/01/2006 - "24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice." 08/01/2008 - "Long-term infantile nephropathic cystinosis can be associated with a form of sclerosing cholangitis, which can respond to therapy with ursodeoxycholic acid." 01/16/2001 - "The association between dysplasia and ursodiol use remained after adjustment for sex, age at onset of colitis, duration of colitis, duration of sclerosing cholangitis, severity of liver disease, and sulfasalazine use (adjusted odds ratio, 0.14 [CI, 0.03 to 0.64]; P = 0.01). "
2.	Immunoglobulin G (IgG)IBA 03/01/2015 - "The final diagnosis was IgG4 sclerosing cholangitis. " 07/01/2013 - "We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy." 07/01/2013 - "Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related sclerosing cholangitis: a Japanese cohort." 03/01/2012 - "IgG4-associated sclerosing cholangitis), which frequently causes problems in clear-cut discrimination from classic PSC and the emerging knowledge about potential disease modifier genes (e.g. " 01/01/2012 - "Case of IgG4-related sclerosing cholangitis with a normal serum IgG4 level: report of a case."
3.	Immunoglobulins (Immunoglobulin)IBA 02/01/2016 - "Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the multisystem IgG4-related disease. " 02/01/2016 - "New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis." 04/01/2015 - "Immunoglobulin G4-related sclerosing cholangitis." 03/01/2015 - "We report a rare case of immunoglobulin G4 (IgG4)-related sclerosing cholangitis without other organ involvement. " 11/01/2014 - "Diagnosis of immunoglobulin g4-related sclerosing cholangitis."
4.	MelatoninIBA 04/01/2010 - "In this experimental study in a rat model of caustic sclerosing cholangitis, the histopathologic and scintigraphic findings suggested that melatonin is effective in attenuating the damage caused by scolicidal agents on the liver and biliary ducts." 04/01/2010 - "Effects of intraperitoneal melatonin on caustic sclerosing cholangitis due to scolicidal solution in a rat model." 04/01/2010 - "Melatonin, an antioxidant, anti-inflammatory, and anticarcinogenic agent, might be used in the treatment of caustic sclerosing cholangitis due to its possible preventive effects on fibrosis and cell damage. " 04/01/2010 - "The aim of the study was to investigate the effects of melatonin on an experimentally developed caustic sclerosing cholangitis with scolicidal solution (formalin) in a rat model. "
5.	Immunoglobulin M (IgM)IBA 06/01/2005 - "Recurrent acalculous cholecystitis and sclerosing cholangitis in a patient with X-linked hyper-immunoglobulin M syndrome." 01/01/2006 - "Long-term (>20 years) intravenous immunoglobulin therapy in 2 patients improved IgG levels but did not fully suppress the high levels of IgM, nor did it prevent late complications (bronchiectasis and sclerosing cholangitis). " 12/01/2013 - "Our patient had high serum IgM with low IgG and IgA levels, lymphocytosis and recurrent infections, intractable diarrhea, growth retardation, systemic CMV infection and sclerosing cholangitis. "
6.	Fluorouracil (Carac)FDA LinkGeneric 06/01/1990 - "Serious chemical sclerosing cholangitis associated with hepatic arterial 5FU and MMC chemotherapy." 08/01/1993 - "Hepatic arterial infusion chemotherapy using 5-FU is effective for colorectal cancer, but there are increasing reports of complications, for example, liver abscess, bile duct necrosis and sclerosing cholangitis. " 06/01/1990 - "A case of iatrogenic sclerosing cholangitis secondary to hepatic intra-arterial 5-fluorouracil (5FU) and Mitomycin C (MMC) chemotherapy is described. " 01/01/2001 - "Sclerosing cholangitis and chemical hepatitis are associated with the use of FUDR, while the use of 5-FU is associated with bone marrow toxicity. "
7.	Factor IX (PTC)FDA LinkGeneric 01/01/1992 - "PTC was particularly helpful in the patient with immunodeficiency in whom features typical of sclerosing cholangitis were found. " 01/01/1994 - "Computed tomography (CT), percutaneous transhepatic cholangiography (PTC) and magnetic resonance imaging (MRI) findings in a case of sclerosing cholangitis associated with Crohn's disease of the colon and terminal ileum are described. " 01/01/1994 - "Crohn's disease and sclerosing cholangitis: CT and PTC diagnosis, with MR findings." 03/01/1990 - "A percutaneous transhepatic cholangiogram (PTC) showed changes typical of sclerosing cholangitis complicated by multiple hepatic abscesses. "
8.	24-norursodeoxycholic acidIBA 02/01/2006 - "24-norUrsodeoxycholic acid ameliorates sclerosing cholangitis in Mdr2(-/-) mice. " 02/01/2006 - "24-norUrsodeoxycholic acid is superior to ursodeoxycholic acid in the treatment of sclerosing cholangitis in Mdr2 (Abcb4) knockout mice."
9.	Primary sclerosing cholangitisIBA 12/01/2015 - "There was a 6% prevalence of primary sclerosing cholangitis (PSC) or autoimmune sclerosing cholangitis (ASC) in this cohort. " 06/01/2015 - "Secondary sclerosing cholangitis has clinical features similar to primary sclerosing cholangitis but originates from a known pathological entity. " 06/01/2015 - "Comparisons of intraductal ultrasonography (IDUS) findings between primary sclerosing cholangitis (PSC) and IgG4-related sclerosing cholangitis (IgG4-SC) have not been elucidated. " 06/01/2015 - "Comparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis." 03/01/2015 - "In 2012 we conducted a nationwide survey for primary sclerosing cholangitis (PSC) and isolated IgG4-related sclerosing cholangitis (IgG4-SC) in Japan, and demonstrated the young as well as the elderly individuals were at higher risk for development of PSC. "
10.	Formaldehyde (Formol)FDA Link 08/01/1990 - "Sclerosing cholangitis induced by formaldehyde solution injected into the biliary tree of rats." 04/01/1989 - "Formalin-induced experimental sclerosing cholangitis in the rat." 01/01/1988 - "Among the various substances utilized, 10% formalin and 30% hypertonic solution caused gross and microscopic lesions compatible with less advanced forms of sclerosing cholangitis. " 02/01/1985 - "100 formalin into normal bile ducts rapidly causes sclerosing cholangitis which often leads to death. " 04/01/1995 - "Secondary sclerosing cholangitis: after injection of formaldehyde into hydatid cysts in the liver."

Therapies and Procedures

1.	Transplants (Transplant) 12/01/2015 - "SSC-CIP was associated with worse prognosis; transplant-free survival was ∼40 months (mean).Because of its high rate of serious complications and unfavorable prognosis, it is imperative to diagnose SSC-CIP early and to differentiate SSC-CIP from other types of sclerosing cholangitis. " 04/01/2008 - "Cryptosporidium parvum-associated sclerosing cholangitis in a liver transplant patient." 05/01/2003 - "parvum-induced sclerosing cholangitis in a renal transplant patient and one of a few in non-HIV patients. " 05/01/2003 - "parvum-induced sclerosing cholangitis in a renal transplant patient. " 02/01/1997 - "Relapse of sclerosing cholangitis after liver transplant in patients with hyper-Ig M syndrome."
2.	Drug Therapy (Chemotherapy) 02/01/2011 - "We diagnosed chemotherapy-induced sclerosing cholangitis (CISC) caused by S-1. " 02/01/2011 - "We report a case of sclerosing cholangitis caused by oral chemotherapy with S-1. " 02/01/2011 - "[A case of sclerosing cholangitis caused by oral chemotherapy with S-1]." 01/01/2009 - "Chemotherapy-induced sclerosing cholangitis as a rare indication for resection: report of a case." 08/01/2006 - "To review the computed tomography (CT), magnetic resonance imaging (MRI) and cholangiographic findings of chemotherapy-induced sclerosing cholangitis (CISC). "
3.	Transplantation (Transplant Recipients) 09/01/2015 - "The second one occurred in a patient who received transplantation for secondary sclerosing cholangitis. " 09/01/1999 - "Sclerosing cholangitis may recur after transplantation, but this has had no clinical implications to date." 02/01/2015 - "When should we consider transplantation in adult patients with sclerosing cholangitis due to multisystem Langerhans' cell histiocytosis?" 05/01/2010 - "A more intense colorectal carcinoma surveillance program based on this result remains controversial in nonprimary sclerosing cholangitis post-liver transplant recipients." 05/01/2010 - "The overall standardized incidence ratio for colorectal carcinoma in post-liver transplant recipients was 2.16 (95% CI: 0.81-5.76) compared with the general population and 1.26 (95% CI: 0.31-5.03) for nonprimary sclerosing cholangitis post-liver transplant recipients. "
4.	Aftercare (After-Treatment) 10/01/2001 - "After treatment, all patients, with the exception of the one with sclerosing cholangitis, remained asymptomatic. " 05/01/2012 - "Herein, we report a case of immunoglobulin G4-related sclerosing cholangitis accompanying autoimmune pancreatitis in a patient who improved with treatment by steroid therapy and endoscopic biliary stenting, and observed the bile duct mucosa by peroral cholangioscopy before and after treatment."
5.	Liver Transplantation 01/01/2015 - "We report on a 60-year-old male patient who developed an extensive multicystic change of the entire pancreas, suspicious for IPMN, under follow-up after liver transplantation for secondary sclerosing cholangitis. " 12/01/2012 - "We report an 18-yr-old girl who had successfully undergone liver transplantation five yr earlier for neonatal sclerosing cholangitis complicated by the development of dysplastic nodules. " 03/01/2012 - "Sclerosing cholangitis, an uncommon disorder in children, is progressive and is, therefore, an important indication for pediatric liver transplantation. " 04/01/2011 - "Outcome of patients with ischemic-like cholangiopathy with secondary sclerosing cholangitis after liver transplantation." 10/01/2009 - "The major complication of post-traumatic damage to the liver is secondary sclerosing cholangitis (SCC) a highly progressive disease for which there is no sufficient therapy except liver transplantation. "