sclerosing cholangitis

Summary

Summary: 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.

Top Publications

  1. Moreno Luna L, Gores G. Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. Liver Transpl. 2006;12:S15-9 pubmed
  2. Shorbagi A, Bayraktar Y. Primary sclerosing cholangitis--what is the difference between east and west?. World J Gastroenterol. 2008;14:3974-81 pubmed
    Primary sclerosing cholangitis (PSC) is a chronic, progressive, cholestatic liver disease characterized by inflammation and fibrotic obliteration of the hepatic biliary tree. It is commonly associated with inflammatory bowel disease (IBD)...
  3. Aron J, Bowlus C. The immunobiology of primary sclerosing cholangitis. Semin Immunopathol. 2009;31:383-97 pubmed publisher
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease histologically characterized by the presence of intrahepatic and/or extrahepatic biliary duct concentric, obliterative fibrosis, eventually leading to cirrhosis...
  4. Hov J, Boberg K, Karlsen T. Autoantibodies in primary sclerosing cholangitis. World J Gastroenterol. 2008;14:3781-91 pubmed
    The aetiology of primary sclerosing cholangitis (PSC) is not known and controversy exists as to whether PSC should be denominated an autoimmune disease...
  5. Bjornsson E, Olsson R, Bergquist A, Lindgren S, Braden B, Chapman R, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology. 2008;134:975-80 pubmed publisher
    The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized...
  6. Treeprasertsuk S, Kowdley K, Luketic V, Harrison M, McCashland T, Befeler A, et al. The predictors of the presence of varices in patients with primary sclerosing cholangitis. Hepatology. 2010;51:1302-10 pubmed publisher
    The predictors for developing varices in patients with primary sclerosing cholangitis (PSC) have not been well studied prospectively...
  7. Hov J, Keitel V, Laerdahl J, Spomer L, Ellinghaus E, ElSharawy A, et al. Mutational characterization of the bile acid receptor TGR5 in primary sclerosing cholangitis. PLoS ONE. 2010;5:e12403 pubmed publisher
    ..linked to inflammatory pathways as well as bile homeostasis, and could therefore be involved in primary sclerosing cholangitis (PSC) a chronic inflammatory bile duct disease...
  8. Etzel J, Eng S, Ko C, Lee S, Saunders M, Tung B, et al. Complications after ERCP in patients with primary sclerosing cholangitis. Gastrointest Endosc. 2008;67:643-8 pubmed
    There are conflicting data regarding the role of ERCP in patients with primary sclerosing cholangitis (PSC) and the risk of procedure-related complications...
  9. Jaeger C, Mayer G, Henrich R, Gossner L, Rabenstein T, May A, et al. Secondary sclerosing cholangitis after long-term treatment in an intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up. Endoscopy. 2006;38:730-4 pubmed
    We present ten patients who developed secondary sclerosing cholangitis following long-term treatment in an intensive care unit (ICU) between 1999 and 2004...

More Information

Publications109 found, 100 shown here

  1. Nyblom H, Nordlinder H, Olsson R. High aspartate to alanine aminotransferase ratio is an indicator of cirrhosis and poor outcome in patients with primary sclerosing cholangitis. Liver Int. 2007;27:694-9 pubmed
    A liver biopsy is performed mainly to stage primary sclerosing cholangitis (PSC)...
  2. Gordon F. Primary sclerosing cholangitis. Surg Clin North Am. 2008;88:1385-407, x pubmed publisher
    Primary sclerosing cholangitis (PSC) is a generally progressive, sometimes fatal chronic hepatobiliary disorder for which no effective medical therapy now exists...
  3. Koyabu M, Uchida K, Fukata N, Kusuda T, Ikeura T, Sakaguchi Y, et al. Primary sclerosing cholangitis with elevated serum IgG4 levels and/or infiltration of abundant IgG4-positive plasma cells. J Gastroenterol. 2010;45:122-9 pubmed publisher
    Immunoglobin G4-related sclerosing cholangitis (IgG4-SC) is recognized as one of the systemic sclerosing diseases characterized by abundant IgG4-positive plasma cells with effective steroid therapy...
  4. Koyabu M, Uchida K, Miyoshi H, Sakaguchi Y, Fukui T, Ikeda H, et al. Analysis of regulatory T cells and IgG4-positive plasma cells among patients of IgG4-related sclerosing cholangitis and autoimmune liver diseases. J Gastroenterol. 2010;45:732-41 pubmed publisher
    ..The most common involved organ showing extrapancreatic lesions is the bile duct, which exhibits sclerosing cholangitis (SC). However, the role of IgG4 in the development of IgG4-related SC (IgG4-SC) remains unclear...
  5. Fevery J, Verslype C, Lai G, Aerts R, Van Steenbergen W. Incidence, diagnosis, and therapy of cholangiocarcinoma in patients with primary sclerosing cholangitis. Dig Dis Sci. 2007;52:3123-35 pubmed
    Primary sclerosing cholangitis (PSC) can lead to the development of cholangiocarcinoma (CCA). The tumor may present as an intrahepatic focal cholangiocellular carcinoma but more often as a ductal infiltrating desmoplastic lesion...
  6. Alazmi W, McHenry L, Watkins J, Fogel E, Schmidt S, Sherman S, et al. Chemotherapy-induced sclerosing cholangitis: long-term response to endoscopic therapy. J Clin Gastroenterol. 2006;40:353-7 pubmed
    ..One major complication of such treatment is biliary sclerosis resembling primary sclerosing cholangitis, which has an incidence ranging from 8% to 26%...
  7. Henkel C, Roderfeld M, Weiskirchen R, Berres M, Hillebrandt S, Lammert F, et al. Changes of the hepatic proteome in murine models for toxically induced fibrogenesis and sclerosing cholangitis. Proteomics. 2006;6:6538-48 pubmed
    We investigated the changes in the hepatic proteome in murine models for toxic-induced fibrogenesis and sclerosing cholangitis. A comprehensive comparison of protein changes observed is made and the mechanistical basis of the expression ..
  8. Wasmuth H, Tranø G, Endreseth B, Wibe A, Rydning A, Myrvold H. Primary sclerosing cholangitis and extraintestinal manifestations in patients with ulcerative colitis and ileal pouch-anal anastomosis. J Gastrointest Surg. 2010;14:1099-104 pubmed publisher
    ..outcomes in patients having ileal pouch-anal anastomosis for ulcerative colitis with or without primary sclerosing cholangitis or extraintestinal manifestations and to assess if primary sclerosing cholangitis is a risk factor for ..
  9. Weismüller T, Wedemeyer J, Kubicka S, Strassburg C, Manns M. The challenges in primary sclerosing cholangitis--aetiopathogenesis, autoimmunity, management and malignancy. J Hepatol. 2008;48 Suppl 1:S38-57 pubmed publisher
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, characterized by progressive inflammation and fibrosis of the bile ducts, resulting in biliary cirrhosis and is associated with a high risk of cholangiocarcinoma...
  10. Claessen M, Vleggaar F, Tytgat K, Siersema P, van Buuren H. High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol. 2009;50:158-64 pubmed publisher
    Primary sclerosing cholangitis (PSC) patients are at risk for developing cholangiocarcinoma (CCA) and colorectal carcinoma (CRC). Our aim was to assess the risk of malignancies and their influence on survival.
  11. Kerkar N, Miloh T. Sclerosing cholangitis: pediatric perspective. Curr Gastroenterol Rep. 2010;12:195-202 pubmed publisher
    b>Sclerosing cholangitis is a rare progressive cholestatic liver disease affecting the biliary tract. It may be associated with other diseases including autoimmune hepatitis, immunodeficiencies, cystic fibrosis, and sickle cell disease...
  12. Fukuhara T, Egashira A, Imamura M, Ikegami T, Oki E, Soejima Y, et al. Proctocolectomy for colon cancer associated with ulcerative colitis a few months after living donor liver transplantation for primary sclerosing cholangitis: report of a case. Surg Today. 2009;39:59-63 pubmed publisher
    ..successfully by proctocolectomy 8 months after living donor liver transplantation (LDLT) for primary sclerosing cholangitis (PSC). The lesion was detected early, probably as a result of colonoscopic surveillance after LDLT...
  13. Boner A, Peroni D, Bodini A, Delaini G, Piacentini G. Azithromycin may reduce cholestasis in primary sclerosing cholangitis: a case report and serendipitous observation. Int J Immunopathol Pharmacol. 2007;20:847-9 pubmed
    ..We report the possible effect of azithromycin on primary sclerosing cholangitis in a patient treated with the drug for severe asthma...
  14. Silveira M, Mendes F, Diehl N, Enders F, Lindor K. Thyroid dysfunction in primary biliary cirrhosis, primary sclerosing cholangitis and non-alcoholic fatty liver disease. Liver Int. 2009;29:1094-100 pubmed publisher
    ..incidence of thyroid dysfunction (TD) in a series of patients with PBC (n=67) with patients with primary sclerosing cholangitis (PSC) (n=79) and non-alcoholic fatty liver disease (NAFLD) (n=97) seen in a tertiary referral centre who ..
  15. Wiencke K, Karlsen T, Boberg K, Thorsby E, Schrumpf E, Lie B, et al. Primary sclerosing cholangitis is associated with extended HLA-DR3 and HLA-DR6 haplotypes. Tissue Antigens. 2007;69:161-9 pubmed
    Primary sclerosing cholangitis (PSC) is associated with the human leukocyte antigen (HLA)-DRB1*0301-DQA1*0501-DQB1*0201 (DR3) and HLA-DRB1*1301-DQA1*0103-DQB1*0603 (DR6) haplotypes...
  16. Kulaksiz H, Heuberger D, Engler S, Stiehl A. Poor outcome in progressive sclerosing cholangitis after septic shock. Endoscopy. 2008;40:214-8 pubmed publisher
    Progressive sclerosing cholangitis after septic shock is an increasingly diagnosed disease entity. We evaluated the outcome after long-term follow-up of 29 patients treated in our institution between 1995 and 2007...
  17. Larusso N, Shneider B, Black D, Gores G, James S, Doo E, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006;44:746-64 pubmed
    Primary sclerosing cholangitis (PSC) is a rare but important liver disease that leads to cirrhosis and need for liver transplantation in a high proportion of cases...
  18. Karlsen T, Lie B, Frey Frøslie K, Thorsby E, Broome U, Schrumpf E, et al. Polymorphisms in the steroid and xenobiotic receptor gene influence survival in primary sclerosing cholangitis. Gastroenterology. 2006;131:781-7 pubmed
    ..of the SXR gene influence disease susceptibility or disease progression in patients with primary sclerosing cholangitis (PSC)...
  19. Pauli Magnus C, Kerb R, Fattinger K, Lang T, Anwald B, Kullak Ublick G, et al. BSEP and MDR3 haplotype structure in healthy Caucasians, primary biliary cirrhosis and primary sclerosing cholangitis. Hepatology. 2004;39:779-91 pubmed
    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are characterized by a cholestatic pattern of liver damage, also observed in hereditary or acquired dysfunction of the canalicular membrane transporters bile salt ..
  20. Lindor K, Kowdley K, Luketic V, Harrison M, McCashland T, Befeler A, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009;50:808-14 pubmed publisher
    ..trials are inconclusive regarding the efficacy of ursodeoxycholic acid (UDCA) for treating primary sclerosing cholangitis (PSC)...
  21. Eapen C, Roberts Thomson I. Primary sclerosing cholangitis. J Gastroenterol Hepatol. 2006;21:1862 pubmed
  22. Miloh T, Arnon R, Shneider B, Suchy F, Kerkar N. A retrospective single-center review of primary sclerosing cholangitis in children. Clin Gastroenterol Hepatol. 2009;7:239-45 pubmed publisher
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and progressive bile duct fibrosis. There are limited data on pediatric PSC...
  23. Plum W, Tschaharganeh D, Kroy D, Corsten E, Erschfeld S, Dierssen U, et al. Lack of glycoprotein 130/signal transducer and activator of transcription 3-mediated signaling in hepatocytes enhances chronic liver injury and fibrosis progression in a model of sclerosing cholangitis. Am J Pathol. 2010;176:2236-46 pubmed publisher
    ..DDC) model leads to chronic cholestatic liver injury and therefore resembles human diseases such as sclerosing cholangitis and forms of metabolic liver diseases...
  24. Melum E, Karlsen T, Broome U, Thorsby E, Schrumpf E, Boberg K, et al. The 32-base pair deletion of the chemokine receptor 5 gene (CCR5-Delta32) is not associated with primary sclerosing cholangitis in 363 Scandinavian patients. Tissue Antigens. 2006;68:78-81 pubmed
    ..Conflicting evidence exists whether this deletion is associated with primary sclerosing cholangitis (PSC). We genotyped the CCR5-Delta32 variant in 363 PSC patients and 366 controls...
  25. Terjung B, Spengler U. Atypical p-ANCA in PSC and AIH: a hint toward a "leaky gut"?. Clin Rev Allergy Immunol. 2009;36:40-51 pubmed publisher
    Primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) are enigmatic chronic inflammatory diseases of the liver, which are frequently associated with chronic inflammatory bowel diseases...
  26. Bjornsson E. Management of primary sclerosing cholangitis. Minerva Gastroenterol Dietol. 2009;55:163-72 pubmed
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with fibrosis surrounding the intrahepatic and/or the extrahepatic bile ducts...
  27. Webster G, Pereira S, Chapman R. Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis--overlapping or separate diseases?. J Hepatol. 2009;51:398-402 pubmed publisher
    ..The cholangiographic appearances of IgG4-associated cholangitis and primary sclerosing cholangitis can be difficult to differentiate...
  28. Bergquist A, Montgomery S, Bahmanyar S, Olsson R, Danielsson A, Lindgren S, et al. Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis. Clin Gastroenterol Hepatol. 2008;6:939-43 pubmed publisher
    The importance of genetic factors for the development of primary sclerosing cholangitis (PSC) is incompletely understood...
  29. Eksteen B, Miles A, Curbishley S, Tselepis C, Grant A, Walker L, et al. Epithelial inflammation is associated with CCL28 production and the recruitment of regulatory T cells expressing CCR10. J Immunol. 2006;177:593-603 pubmed
    ..We propose that CXCR3 promotes the recruitment of Tregs to inflamed tissues and CCR10 allows them to respond to CCL28 secreted by epithelial cells resulting in the accumulation of CCR10+ Tregs at mucosal surfaces. ..
  30. Bjornsson E, Chari S, Smyrk T, Lindor K. Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology. 2007;45:1547-54 pubmed
  31. Silveira M, Lindor K. Primary sclerosing cholangitis. Can J Gastroenterol. 2008;22:689-98 pubmed
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy...
  32. Ruemmele P, Hofstaedter F, Gelbmann C. Secondary sclerosing cholangitis. Nat Rev Gastroenterol Hepatol. 2009;6:287-95 pubmed publisher
    Secondary sclerosing cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation and progressive destruction of the biliary tree that leads to ..
  33. Fickert P, Moustafa T, Trauner M. Primary sclerosing cholangitis--the arteriosclerosis of the bile duct?. Lipids Health Dis. 2007;6:3 pubmed
    Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of unknown aetiology affecting the large bile ducts and characterized by periductal fibrosis and stricture formation, which ultimately result in biliary cirrhosis and ..
  34. Krasinskas A, Yao Y, Randhawa P, Dore M, Sepulveda A. Helicobacter pylori may play a contributory role in the pathogenesis of primary sclerosing cholangitis. Dig Dis Sci. 2007;52:2265-70 pubmed
    ..To better understand the role of H pylori in primary sclerosing cholangitis (PSC), 25 patients with end-stage PSC and 31 controls were studied...
  35. Melum E, Karlsen T, Schrumpf E, Bergquist A, Thorsby E, Boberg K, et al. Cholangiocarcinoma in primary sclerosing cholangitis is associated with NKG2D polymorphisms. Hepatology. 2008;47:90-6 pubmed
    Primary sclerosing cholangitis (PSC) is often complicated by the development of cholangiocarcinoma (CCA). Genetic variation of natural killer cell receptor G2D (NKG2D) has been associated with cancer susceptibility...
  36. Henckaerts L, Fevery J, Van Steenbergen W, Verslype C, Yap P, Nevens F, et al. CC-type chemokine receptor 5-Delta32 mutation protects against primary sclerosing cholangitis. Inflamm Bowel Dis. 2006;12:272-7 pubmed
    Primary sclerosing cholangitis (PSC) is commonly associated with inflammatory bowel disease (IBD) and characterized by fibrosing inflammatory destruction of biliary ducts...
  37. Kaplan G, Laupland K, Butzner D, Urbanski S, Lee S. The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis. Am J Gastroenterol. 2007;102:1042-9 pubmed
    The epidemiology of primary sclerosing cholangitis (PSC) has been incompletely assessed by population-based studies...
  38. Karlsen T, Schrumpf E, Boberg K. Update on primary sclerosing cholangitis. Dig Liver Dis. 2010;42:390-400 pubmed publisher
    Early studies in primary sclerosing cholangitis (PSC) were concerned with disease characterization, and were followed by epidemiological studies of PSC and clinical subsets of PSC as well as a large number of treatment trials...
  39. Walker S, McCormick P. Diagnosing cholangiocarcinoma in primary sclerosing cholangitis: an "evidence based radiology" review. Abdom Imaging. 2008;33:14-7 pubmed
    Cholangiocarcinoma is a serious but common complication of primary sclerosing cholangitis (PSC) that is often difficult to diagnose...
  40. Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41:626-31 pubmed
  41. Fickert P, Stöger U, Fuchsbichler A, Moustafa T, Marschall H, Weiglein A, et al. A new xenobiotic-induced mouse model of sclerosing cholangitis and biliary fibrosis. Am J Pathol. 2007;171:525-36 pubmed
    ..This model may be valuable to investigate the mechanisms of xenobiotic-induced chronic cholangiopathies and its sequels including biliary fibrosis...
  42. Miura F, Asano T, Amano H, Yoshida M, Toyota N, Wada K, et al. Resected case of eosinophilic cholangiopathy presenting with secondary sclerosing cholangitis. World J Gastroenterol. 2009;15:1394-7 pubmed
    ..is a rare condition characterized by eosinophilic infiltration of the biliary tract and causes sclerosing cholangitis. We report a patient with secondary sclerosing cholangitis with eosinophilic cholecystitis...
  43. Kashyap R, Mantry P, Sharma R, Maloo M, Safadjou S, Qi Y, et al. Comparative analysis of outcomes in living and deceased donor liver transplants for primary sclerosing cholangitis. J Gastrointest Surg. 2009;13:1480-6 pubmed publisher
    ..We present a single-center experience on survival outcomes and disease recurrence for LDLT and DDLT for ESLD secondary to PSC...
  44. Kamisawa T, Takuma K, Anjiki H, Egawa N, Kurata M, Honda G, et al. Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis. World J Gastroenterol. 2009;15:2357-60 pubmed
    To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis (AIP) and compare them with those of primary sclerosing cholangitis (PSC).
  45. Kato Y, Matsubara K, Akiyama Y, Hattori H, Hirata A, Suzuki F, et al. Chemotherapy-induced sclerosing cholangitis as a rare indication for resection: report of a case. Surg Today. 2009;39:905-8 pubmed publisher
    Bile duct stricture due to chemotherapy-induced sclerosing cholangitis (CISC) is a potentially fatal complication of hepatic arterial infusion chemotherapy (HAIC). It is managed primarily with medical treatment and biliary stenting...
  46. Duclos Vallee J, Sebagh M. Recurrence of autoimmune disease, primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune hepatitis after liver transplantation. Liver Transpl. 2009;15 Suppl 2:S25-34 pubmed publisher
    1. Recurrence of primary sclerosing cholangitis. a Five percent of liver transplants are performed because of end-stage liver disease secondary to primary sclerosing cholangitis. b Recurrent disease affects 10% to 27% of recipients...
  47. Baghdasaryan A, Claudel T, Kosters A, Gumhold J, Silbert D, Thüringer A, et al. Curcumin improves sclerosing cholangitis in Mdr2-/- mice by inhibition of cholangiocyte inflammatory response and portal myofibroblast proliferation. Gut. 2010;59:521-30 pubmed publisher
    ..Curcumin, the yellow pigment of the spice turmeric, has pleiotropic actions and attenuates hepatic damage in animal models of chemically-induced liver injury. Whether curcumin has beneficial effects in cholangiopathies is unknown...
  48. Maggs J, Chapman R. Sclerosing cholangitis. Curr Opin Gastroenterol. 2007;23:310-6 pubmed
    Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree complicated by cirrhosis and cholangiocarcinoma. It is immune mediated, although the precise aetiology remains unknown.
  49. Zen Y, Fujii T, Harada K, Kawano M, Yamada K, Takahira M, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology. 2007;45:1538-46 pubmed
    ..7-fold), IL-10/CD4 (45.3-fold), and tumor growth factor (TGF)-beta/CD4 (39.4-fold) than did primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC)...
  50. Pall H, Zielenski J, Jonas M, DaSilva D, Potvin K, Yuan X, et al. Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J Pediatr. 2007;151:255-9 pubmed
    To determine whether primary sclerosing cholangitis (PSC) in childhood is associated with abnormalities in cystic fibrosis transmembrane conductance regulator (CFTR).
  51. Schnitzbauer A, Tsui T, Kirchner G, Scherer M, Bein T, Schlitt H, et al. Liver transplantation for sclerosing cholangitis in a polytraumatized patient. Nat Clin Pract Gastroenterol Hepatol. 2009;6:121-6 pubmed publisher
    ..The patient developed acute liver failure 8 weeks after the initial trauma...
  52. Olsson R, Glaumann H, Almer S, Broome U, Lebrun B, Bergquist A, et al. High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis. Eur J Intern Med. 2009;20:190-6 pubmed publisher
    Overlap syndrome is a term used for overlapping features of autoimmune hepatitis and primary sclerosing cholangitis or primary biliary cirrhosis and for autoimmune cholangitis...
  53. Mieli Vergani G, Vergani D. Unique features of primary sclerosing cholangitis in children. Curr Opin Gastroenterol. 2010;26:265-8 pubmed publisher
    To summarize publications on juvenile primary sclerosing cholangitis (PSC) published over the past 5 years...
  54. Bowlus C, Karlsen T, Broome U, Thorsby E, Vatn M, Schrumpf E, et al. Analysis of MAdCAM-1 and ICAM-1 polymorphisms in 365 Scandinavian patients with primary sclerosing cholangitis. J Hepatol. 2006;45:704-10 pubmed
    ..MAdCAM-1) has been implicated in the aberrant homing of intestinal lymphocytes to the liver in primary sclerosing cholangitis (PSC)...
  55. Moff S, Kamel I, Eustace J, Lawler L, Kantsevoy S, Kalloo A, et al. Diagnosis of primary sclerosing cholangitis: a blinded comparative study using magnetic resonance cholangiography and endoscopic retrograde cholangiography. Gastrointest Endosc. 2006;64:219-23 pubmed
    We hypothesized that magnetic resonance cholangiography (MRC) may have less accuracy for the diagnosis and the assessment of the severity of primary sclerosing cholangitis (PSC) than endoscopic retrograde cholangiography (ERC).
  56. Maggs J, Chapman R. An update on primary sclerosing cholangitis. Curr Opin Gastroenterol. 2008;24:377-83 pubmed publisher
    Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by strictures of the biliary tree complicated by cirrhosis and cholangiocarcinoma. It is immune mediated, although the precise aetiology remains unknown.
  57. Tischendorf J, Geier A, Trautwein C. Current diagnosis and management of primary sclerosing cholangitis. Liver Transpl. 2008;14:735-46 pubmed publisher
    Primary sclerosing cholangitis (PSC) is an important liver disease with major morbidity and mortality...
  58. Davies Y, Cox K, Abdullah B, Safta A, Terry A, Cox K. Long-term treatment of primary sclerosing cholangitis in children with oral vancomycin: an immunomodulating antibiotic. J Pediatr Gastroenterol Nutr. 2008;47:61-7 pubmed publisher
    Primary sclerosing cholangitis is a rare chronic cholestatic condition of unknown etiology, frequently associated with inflammatory bowel disease and characterized by diffuse fibrosing and inflammatory destruction of the intra- and/or ..
  59. Alabraba E, Nightingale P, Gunson B, Hubscher S, Olliff S, Mirza D, et al. A re-evaluation of the risk factors for the recurrence of primary sclerosing cholangitis in liver allografts. Liver Transpl. 2009;15:330-40 pubmed publisher
    ..that the absence of the colon after liver transplantation (LT) protects the patient from recurrent primary sclerosing cholangitis (rPSC)...
  60. Lepistö A, Kivistö S, Kivisaari L, Arola J, Jarvinen H. Primary sclerosing cholangitis: outcome of patients undergoing restorative proctocolecetomy for ulcerative colitis. Int J Colorectal Dis. 2009;24:1169-74 pubmed publisher
    The prevalence of primary sclerosing cholangitis (PSC) among patients with ulcerative colitis needing proctocolectomy is about 12%. The study aim was to evaluate the progression of the liver disease after surgery.
  61. Hov J, Lleo A, Selmi C, Woldseth B, Fabris L, Strazzabosco M, et al. Genetic associations in Italian primary sclerosing cholangitis: heterogeneity across Europe defines a critical role for HLA-C. J Hepatol. 2010;52:712-7 pubmed publisher
    The HLA complex on chromosome 6p21 is firmly established as a risk locus for primary sclerosing cholangitis (PSC)...
  62. Sinakos E, Marschall H, Kowdley K, Befeler A, Keach J, Lindor K. Bile acid changes after high-dose ursodeoxycholic acid treatment in primary sclerosing cholangitis: Relation to disease progression. Hepatology. 2010;52:197-203 pubmed publisher
    ..mg/kg/day) ursodeoxycholic acid (UDCA) treatment improves serum liver tests in patients with primary sclerosing cholangitis (PSC) but does not improve survival and is associated with increased rates of serious adverse events...
  63. Gelbmann C, Rümmele P, Wimmer M, Hofstadter F, Göhlmann B, Endlicher E, et al. Ischemic-like cholangiopathy with secondary sclerosing cholangitis in critically ill patients. Am J Gastroenterol. 2007;102:1221-9 pubmed
    b>Sclerosing cholangitis in critically ill patients (SC-CIP) is a newly described entity of severe biliary disease with progression to liver cirrhosis...
  64. Esposito I, Kubisova A, Stiehl A, Kulaksiz H, Schirmacher P. Secondary sclerosing cholangitis after intensive care unit treatment: clues to the histopathological differential diagnosis. Virchows Arch. 2008;453:339-45 pubmed publisher
    Secondary sclerosing cholangitis (SSC) is a chronic cholestatic disorder caused by mechanical, infectious, toxic, or ischemic factors...
  65. Rudolph G, Gotthardt D, Klöters Plachky P, Kulaksiz H, Rost D, Stiehl A. Influence of dominant bile duct stenoses and biliary infections on outcome in primary sclerosing cholangitis. J Hepatol. 2009;51:149-55 pubmed publisher
    In primary sclerosing cholangitis (PSC) dominant stenoses are frequently associated with bacterial, and in part, also fungal infections of the bile ducts...
  66. Miura H, Miyachi Y. IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis. A recurrent case after a 5-year history of spontaneous remission. JOP. 2009;10:432-7 pubmed
    ..This report describes the case of IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis, which recurred after a long period of spontaneous remission.
  67. Deshpande V, Sainani N, Chung R, Pratt D, Mentha G, Rubbia Brandt L, et al. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Mod Pathol. 2009;22:1287-95 pubmed publisher
    ..condition associated with autoimmune pancreatitis that clinically and radiologically mimics primary sclerosing cholangitis. In this study, we conducted a morphological and immunohistochemical analysis of liver material obtained ..
  68. Naitoh I, Nakazawa T, Ohara H, Ando T, Hayashi K, Tanaka H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis. J Gastroenterol. 2009;44:1147-55 pubmed publisher
    IgG4-related sclerosing cholangitis (IgG4-SC) is one of the diseases associated with autoimmune pancreatitis. Several cases of IgG4-SC showed no pancreas abnormalities and it was difficult to distinguish cholangiocarcinoma...
  69. Karlsen T, Franke A, Melum E, Kaser A, Hov J, Balschun T, et al. Genome-wide association analysis in primary sclerosing cholangitis. Gastroenterology. 2010;138:1102-11 pubmed publisher
    We aimed to characterize the genetic susceptibility to primary sclerosing cholangitis (PSC) by means of a genome-wide association analysis of single nucleotide polymorphism (SNP) markers.
  70. Zein C, Lindor K. Latest and emerging therapies for primary biliary cirrhosis and primary sclerosing cholangitis. Curr Gastroenterol Rep. 2010;12:13-22 pubmed publisher
    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two most common causes of chronic cholestatic liver disease in adults...
  71. Karlsen T, Schrumpf E, Boberg K. Genetic epidemiology of primary sclerosing cholangitis. World J Gastroenterol. 2007;13:5421-31 pubmed
    The aetiology of primary sclerosing cholangitis (PSC) is not known. A more than 80-fold increased risk of PSC among first-degree relatives emphasizes the importance of genetic factors...
  72. Card T, Solaymani Dodaran M, West J. Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study. J Hepatol. 2008;48:939-44 pubmed publisher
    Little is known about the occurrence of Primary Sclerosing Cholangitis (PSC) in the population of the United Kingdom or its associated risks of mortality and malignancy. We aimed to fill these gaps in knowledge.
  73. Gaj P, Habior A, Mikula M, Ostrowski J. Lack of evidence for association of primary sclerosing cholangitis and primary biliary cirrhosis with risk alleles for Crohn's disease in Polish patients. BMC Med Genet. 2008;9:81 pubmed publisher
    ..We investigated whether polymorphisms that confer susceptibility to Crohn's disease could be classified also as predisposing factors for the development of primary sclerosing cholangitis and primary biliary cirrhosis in Polish patients.
  74. Forsbring M, Vik E, Dalhus B, Karlsen T, Bergquist A, Schrumpf E, et al. Catalytically impaired hMYH and NEIL1 mutant proteins identified in patients with primary sclerosing cholangitis and cholangiocarcinoma. Carcinogenesis. 2009;30:1147-54 pubmed publisher
    ..Primary sclerosing cholangitis (PSC), a chronic cholestatic liver disease characterized by inflammatory destruction of the biliary tree, ..
  75. Itoh S, Nagasaka T, Suzuki K, Satake H, Ota T, Naganawa S. Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clin Radiol. 2009;64:1104-14 pubmed publisher
    To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis.
  76. Mair M, Zollner G, Schneller D, Musteanu M, Fickert P, Gumhold J, et al. Signal transducer and activator of transcription 3 protects from liver injury and fibrosis in a mouse model of sclerosing cholangitis. Gastroenterology. 2010;138:2499-508 pubmed publisher
    ..3 (Stat3) is the main mediator of interleukin-6-type cytokine signaling required for hepatocyte proliferation and hepatoprotection, but its role in sclerosing cholangitis and other cholestatic liver diseases remains unresolved.
  77. Ozin Y, Parlak E, Kilic Z, Temucin T, Sasmaz N. Sclerosing cholangitis-like changes in hepatobiliary tuberculosis. Turk J Gastroenterol. 2010;21:50-3 pubmed
    ..irregularities, strictures and dilatations both in the intra- and extrahepatic bile ducts resembling sclerosing cholangitis. The liver biopsy showed caseous granulomatous hepatitis and Langhans giant cells compatible with ..
  78. Nagtzaam I, van Geel M, Driessen A, Steijlen P, van Steensel M. Bile duct paucity is part of the neonatal ichthyosis-sclerosing cholangitis phenotype. Br J Dermatol. 2010;163:205-7 pubmed publisher
    Neonatal ichthyosis-sclerosing cholangitis (NISCh) syndrome is a rare autosomal recessive disorder associated with scalp hypotrichosis, scarring alopecia, ichthyosis and sclerosing cholangitis...
  79. Dave M, Elmunzer B, Dwamena B, Higgins P. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology. 2010;256:387-96 pubmed publisher
    To determine the diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) for detection of primary sclerosing cholangitis (PSC) in patients with biochemical cholestasis.
  80. Tischendorf J, Hecker H, Krüger M, Manns M, Meier P. Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study. Am J Gastroenterol. 2007;102:107-14 pubmed
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with varying severity and progression...
  81. Murray M, Burton F, Di Bisceglie A. Markedly elevated serum CA 19-9 levels in association with a benign biliary stricture due to primary sclerosing cholangitis. J Clin Gastroenterol. 2007;41:115-7 pubmed
    ..This led to a diagnosis of primary sclerosing cholangitis being made, with a dominant stricture in the common bile duct...
  82. Lewis J, Talwalkar J, Rosen C, Smyrk T, Abraham S. Prevalence and risk factors for gallbladder neoplasia in patients with primary sclerosing cholangitis: evidence for a metaplasia-dysplasia-carcinoma sequence. Am J Surg Pathol. 2007;31:907-13 pubmed
    Primary sclerosing cholangitis (PSC) carries an increased risk (10% to 20%) of hepatobiliary malignancy, especially cholangiocarcinoma (CC)...
  83. Mueller T, Bianchi L, Menges M. Autoimmune hepatitis 2 years after the diagnosis of primary sclerosing cholangitis: an unusual overlap syndrome in a 17-year-old adolescent. Eur J Gastroenterol Hepatol. 2008;20:232-6 pubmed publisher
    ..The endoscopic retrograde cholangiography documented typical signs of primary sclerosing cholangitis with involvement of the small and large ducts...
  84. Beuers U, Kullak Ublick G, Pusl T, Rauws E, Rust C. Medical treatment of primary sclerosing cholangitis: a role for novel bile acids and other (post-)transcriptional modulators?. Clin Rev Allergy Immunol. 2009;36:52-61 pubmed publisher
    Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic disease of the liver and bile ducts that is associated with inflammatory bowel disease, generally leads to end-stage liver disease, and is complicated by malignancies of ..
  85. Oh H, Kim J, Kim J, Lee K, Kim M, Chi K, et al. Early bile duct cancer in a background of sclerosing cholangitis and autoimmune pancreatitis. Intern Med. 2008;47:2025-8 pubmed
    ..and cholestasis are among bile duct cancer risk factors, the cause-and-effect relationship between sclerosing cholangitis and bile duct cancer needs to be determined by further study, as between AIP and pancreatic cancer...
  86. Silveira M, Torok N, Gossard A, Keach J, Jorgensen R, Petz J, et al. Minocycline in the treatment of patients with primary sclerosing cholangitis: results of a pilot study. Am J Gastroenterol. 2009;104:83-8 pubmed publisher
    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of young adults that is associated with significant morbidity and mortality. No effective medical therapy is available...
  87. Landaverde C, Ng V, Sato A, Tabibian J, Durazo F, Busuttil R. De-novo cholangiocarcinoma in native common bile duct remnant following OLT for primary sclerosing cholangitis. Ann Hepatol. 2009;8:379-83 pubmed
    Primary sclerosing cholangitis (PSC) is a chronic, progressive, inflammatory and obstructive disease of the intra- and extra-hepatic bile ducts of unknown etiology...
  88. Tada S, Ebinuma H, Saito H, Hibi T. Therapeutic benefit of sulfasalazine for patients with primary sclerosing cholangitis. J Gastroenterol. 2006;41:388-9 pubmed
  89. Tischendorf J, Meier P, Strassburg C, Klempnauer J, Hecker H, Manns M, et al. Characterization and clinical course of hepatobiliary carcinoma in patients with primary sclerosing cholangitis. Scand J Gastroenterol. 2006;41:1227-34 pubmed
    Primary sclerosing cholangitis (PSC) confers a high risk of development of hepatobiliary carcinoma (HBC). The aim of the study was to identify indicators and risk factors for developing HBC in PSC patients.
  90. Karlsen T, Hampe J, Wiencke K, Schrumpf E, Thorsby E, Lie B, et al. Genetic polymorphisms associated with inflammatory bowel disease do not confer risk for primary sclerosing cholangitis. Am J Gastroenterol. 2007;102:115-21 pubmed
    Approximately 80% of patients with primary sclerosing cholangitis (PSC) of Northern European origin have concurrent inflammatory bowel disease (IBD)...
  91. Leung U, Wong P, Roberts R, Koea J. Gall bladder polyps in sclerosing cholangitis: does the 1-cm rule apply?. ANZ J Surg. 2007;77:355-7 pubmed
    ..In the general population PLG less than 1 cm in diameter are thought to have a low risk of malignancy and can be cautiously observed...
  92. Ardesjö B, Hansson C, Bruder C, Rorsman F, Betterle C, Dumanski J, et al. Autoantibodies to glutathione S-transferase theta 1 in patients with primary sclerosing cholangitis and other autoimmune diseases. J Autoimmun. 2008;30:273-82 pubmed publisher
    Primary sclerosing cholangitis (PSC) is an enigmatic disorder with a suggested autoimmune basis. A variety of autoantigens have been suggested but no specific or highly directed epitope has been identified...