Materiały źródłowe

• #1 Primary sclerosing cholangitis in adults: Management – UpToDate

  https://www.uptodate.com/contents/primary-sclerosing-cholangitis-in-adults-management

  Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown etiology that is characterized by inflammation, fibrosis, and stricturing of medium and large-size ducts in the intrahepatic and extrahepatic biliary tree. […] There are two major goals of treatment in PSC: […] Retardation and reversal of the disease process […] Management of progressive disease and its complications.

• #2 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #3

  https://journals.lww.com/ajg/fulltext/2020/06000/targeting_the_gut_microbiome_as_a_treatment_for.9.aspx

  Primary sclerosing cholangitis (PSC) is a rare, immune-mediated, chronic cholestatic liver disease associated with a unique phenotype of inflammatory bowel disease that frequently manifests as pancolitis with right-sided predominance. […] Currently, orthoptic liver transplantation is the only established life-saving treatment, whereas antimicrobial therapy or fecal transplantation is an emerging therapeutic option for PSC. […] A recent Clinical Guideline of the American College of Gastroenterology concludes that there is no approved or proven therapy for PSC. On the other hand, there is some evidence that antibiotic therapy is beneficial in patients with PSC. […] Overall, alterations of the gastrointestinal microbiome by antibiotic therapy seem to alter the natural course of PSC and delay the progression of disease. It might even be speculated that favorable permanent changes of the gastrointestinal microbiome could potentially cure PSC.

• #4 Primary sclerosing cholangitis in adults: Management – UpToDate

  https://www.uptodate.com/contents/primary-sclerosing-cholangitis-in-adults-management

  Primary sclerosing cholangitis (PSC) is a chronic progressive disorder of unknown etiology that is characterized by inflammation, fibrosis, and stricturing of medium and large-size ducts in the intrahepatic and extrahepatic biliary tree. […] There are two major goals of treatment in PSC: […] Retardation and reversal of the disease process […] Management of progressive disease and its complications.

• #5 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #6 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Currently, there is no treatment that can slow or stop primary sclerosing cholangitis from progressing. You can treat some of the symptoms and complications directly. For example, your healthcare provider may prescribe: […] Your healthcare provider will also keep your liver and bile ducts under surveillance. As the disease progresses, they may be able to periodically intervene by opening up a blocked bile duct. They can do this through an ERCP (endoscopic retrograde cholangiopancreatography), a type of exam that allows access to your bile ducts without surgery. […] This is a temporary solution, however. Over the course of 10 to 20 years, primary sclerosing cholangitis eventually progresses from early to late-stage liver disease, and finally, liver failure. Your healthcare provider will monitor your liver damage to determine when you should begin to consider liver transplantation. You’ll need to pass certain qualifications to get on a liver transplantation waiting list.

• #7 Primary sclerosing cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/diagnosis-treatment/drc-20355802

  Treatments for primary sclerosing cholangitis focus on managing complications and monitoring liver damage. Many medications have been studied in people with primary sclerosing cholangitis, but so far none have been found to slow or reverse the liver damage associated with this disease. […] Bile acid sequestrants. Medications that bind to bile acids the substances thought to cause itching in liver disease are the first line treatment for itching in primary sclerosing cholangitis. […] A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. […] A liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis. Though uncommon, it’s possible for primary sclerosing cholangitis to recur after a liver transplant.

• #8 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #9 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Currently, there is no treatment that can slow or stop primary sclerosing cholangitis from progressing. You can treat some of the symptoms and complications directly. For example, your healthcare provider may prescribe: […] Your healthcare provider will also keep your liver and bile ducts under surveillance. As the disease progresses, they may be able to periodically intervene by opening up a blocked bile duct. They can do this through an ERCP (endoscopic retrograde cholangiopancreatography), a type of exam that allows access to your bile ducts without surgery. […] This is a temporary solution, however. Over the course of 10 to 20 years, primary sclerosing cholangitis eventually progresses from early to late-stage liver disease, and finally, liver failure. Your healthcare provider will monitor your liver damage to determine when you should begin to consider liver transplantation. You’ll need to pass certain qualifications to get on a liver transplantation waiting list.

• #10 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #11 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #12 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Medical treatment is directed both at modifying the course of the disease and at symptomatic relief. […] Surgical therapy currently is limited to liver transplantation. […] Ursodeoxycholic acid (UDCA) is the most commonly used drug to treat PSC. […] UDCA has a choleretic effect that increases bile flow and thus excretion of potentially toxic bile acids and other metabolites. […] The only controlled trial compared prednisone, in combination with colchicine, to concurrent historical controls. […] Corticosteroids can be beneficial in patients who have been diagnosed as having a PSC/autoimmune hepatitis overlap syndrome. […] Liver transplantation is the only form of therapy that improves survival in PSC patients who develop end-stage liver disease. […] Treatment of PSC remains a work in progress. […] Among disease-modifying agents, UDCA is the most promising. […] Endoscopic therapy is the approach of choice for dominant strictures. […] For patients with end-stage liver disease due to PSC, liver transplantation is the only therapy that improves survival.

• #13 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Medical treatment is directed both at modifying the course of the disease and at symptomatic relief. […] Surgical therapy currently is limited to liver transplantation. […] Ursodeoxycholic acid (UDCA) is the most commonly used drug to treat PSC. […] UDCA has a choleretic effect that increases bile flow and thus excretion of potentially toxic bile acids and other metabolites. […] The only controlled trial compared prednisone, in combination with colchicine, to concurrent historical controls. […] Corticosteroids can be beneficial in patients who have been diagnosed as having a PSC/autoimmune hepatitis overlap syndrome. […] Liver transplantation is the only form of therapy that improves survival in PSC patients who develop end-stage liver disease. […] Treatment of PSC remains a work in progress. […] Among disease-modifying agents, UDCA is the most promising. […] Endoscopic therapy is the approach of choice for dominant strictures. […] For patients with end-stage liver disease due to PSC, liver transplantation is the only therapy that improves survival.

• #14 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  Use of ursodeoxycholic acid (UDCA) is debatable and controversial. The American Association for the Study of Liver Diseases (AASLD) does not recommend UDCA for as a routine chemopreventive agent to reduce the risk of colorectal dysplasia in patients with PSC and ulcerative colitis; the European Association for the Study of the Liver (EASL) recommends oral UDCA (13-15 mg/kg/day) as first-line pharmacotherapy in all patients with PSC, often continued for life. […] UDCA has been shown to improve the liver function profile in some patients and, in conjunction with endoscopic dilation, has shown a survival benefit in some studies. […] Indications for liver transplantation in patients with primary sclerosing cholangitis (PSC) include variceal bleeding or portal gastropathy, intractable ascites, recurrent cholangitis, progressive muscle wasting, hepatic encephalopathy, and poor quality of life.

• #15 Primary Sclerosing Cholangitis (PSC) – Guts UK

  https://gutscharity.org.uk/advice-and-information/conditions/primary-sclerosing-cholangitis-psc/

  What treatment is available for PSC? Liver transplantation is currently the only life-extending treatment for PSC, and no medicine has been shown to slow disease progression. However, there are several clinical trials underway to evaluate potential new treatments. […] Ursodeoxycholic acid (UDCA) is sometimes prescribed for people with PSC. This medicine has been shown to improve liver blood tests in some people. However, there is no evidence that it slows the rate of liver disease, reduces cancer risk, or prevents a need for transplantation. It can be helpful to talk to your doctor about the risks and benefits of UDCA at low to moderate doses (i.e. between 17-22 milligrams of UDCA for every kg of body weight you are). Higher doses can lead to complications and should be avoided. […] In some individuals, a procedure called an ERCP may be performed. This treatment is used to widen narrowings in the bile ducts. However, for most people with PSC several bile ducts are affected and this approach is not effective. It can be helpful to talk to your doctor about the risks and benefits of undergoing an ERCP.

• #16 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  While UDCA (15 mg/kg/day) is considered the first-line treatment for PBC, its effectiveness in PSC patients is less clear. A randomized, placebo-controlled trial involving 105 PSC patients who received 13 to 15 mg/kg/day of UDCA or a placebo showed a significant reduction in ALP levels, although there were no significant effects on histological progression or the time to liver transplantation. […] The effects of higher doses of UDCA (28 to 30 mg/kg/day) were assessed in a long-term, 5-year, randomized, double-blind, controlled trial. Although this higher dosage significantly lowered serum ALP and ALT levels, the study concluded that serious adverse events were more common in the UDCA than placebo group. […] NorUDCA has demonstrated anti-inflammatory, anti-fibrotic, and anti-proliferative effects in preclinical models of PSC. It has also been shown to lower serum levels of ALP, γGT, AST, and ALT in phase 2 trials involving PBC patients, with a phase 3 trial currently underway to assess its impact on histological progression. […] OCA has been approved as a second-line add-on therapy to UDCA in PBC due to evidence of biochemical efficacy in a phase III study. In PSC patients, 5–10 mg OCA once a day for 24 weeks was found to be partially effective in reducing ALP levels, with no change in fibrosis markers.

• #17 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  Use of ursodeoxycholic acid (UDCA) is debatable and controversial. The American Association for the Study of Liver Diseases (AASLD) does not recommend UDCA for as a routine chemopreventive agent to reduce the risk of colorectal dysplasia in patients with PSC and ulcerative colitis; the European Association for the Study of the Liver (EASL) recommends oral UDCA (13-15 mg/kg/day) as first-line pharmacotherapy in all patients with PSC, often continued for life. […] UDCA has been shown to improve the liver function profile in some patients and, in conjunction with endoscopic dilation, has shown a survival benefit in some studies. […] Indications for liver transplantation in patients with primary sclerosing cholangitis (PSC) include variceal bleeding or portal gastropathy, intractable ascites, recurrent cholangitis, progressive muscle wasting, hepatic encephalopathy, and poor quality of life.

• #18 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #19 Treatment of primary sclerosing cholangitis combined with inflammatory bowel disease

  https://irjournal.org/journal/view.php?number=1015

  Several potential candidate drugs are undergoing clinical trials. […] Despite PSC-IBD is classified as an immune-mediated disease, immunosuppressant treatments have proven to be ineffective. […] Currently, UDCA is the most widely used drug; however, the role of UDCA in the treatment of PSC remains controversial. […] In particular, bile acids modulators appear to be promising agents and have safety profile in terms of IBD activity. Combination therapy may be probably needed to achieve clinical efficacy.

• #20 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Medical treatment is directed both at modifying the course of the disease and at symptomatic relief. […] Surgical therapy currently is limited to liver transplantation. […] Ursodeoxycholic acid (UDCA) is the most commonly used drug to treat PSC. […] UDCA has a choleretic effect that increases bile flow and thus excretion of potentially toxic bile acids and other metabolites. […] The only controlled trial compared prednisone, in combination with colchicine, to concurrent historical controls. […] Corticosteroids can be beneficial in patients who have been diagnosed as having a PSC/autoimmune hepatitis overlap syndrome. […] Liver transplantation is the only form of therapy that improves survival in PSC patients who develop end-stage liver disease. […] Treatment of PSC remains a work in progress. […] Among disease-modifying agents, UDCA is the most promising. […] Endoscopic therapy is the approach of choice for dominant strictures. […] For patients with end-stage liver disease due to PSC, liver transplantation is the only therapy that improves survival.

• #21 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Medical treatment is directed both at modifying the course of the disease and at symptomatic relief. […] Surgical therapy currently is limited to liver transplantation. […] Ursodeoxycholic acid (UDCA) is the most commonly used drug to treat PSC. […] UDCA has a choleretic effect that increases bile flow and thus excretion of potentially toxic bile acids and other metabolites. […] The only controlled trial compared prednisone, in combination with colchicine, to concurrent historical controls. […] Corticosteroids can be beneficial in patients who have been diagnosed as having a PSC/autoimmune hepatitis overlap syndrome. […] Liver transplantation is the only form of therapy that improves survival in PSC patients who develop end-stage liver disease. […] Treatment of PSC remains a work in progress. […] Among disease-modifying agents, UDCA is the most promising. […] Endoscopic therapy is the approach of choice for dominant strictures. […] For patients with end-stage liver disease due to PSC, liver transplantation is the only therapy that improves survival.

• #22 Primary sclerosing cholangitis: Symptoms, causes, diagnosis

  https://www.medicalnewstoday.com/articles/190952

  Treatment includes ursodiol, or ursodeoxycholic acid, a bile acid naturally produced by the liver. […] Research shows that it can lower elevated liver enzyme levels in people with PSC. However, it does not improve the liver’s condition or overall survival rates. […] A doctor may also prescribe: medications to relieve itching, called antipruritics; bile acid sequestrants, such as cholestyramine, to reduce cholesterol; antibiotics to treat infections; vitamin supplements. […] If a person also has ulcerative colitis, the doctor will recommend appropriate treatment. […] If fluid retention from cirrhosis results in swelling in the abdomen and feet, a low-salt diet and diuretics may help. […] Researchers have tried using various immunosuppressants and antibiotics to manage PSC, but these have had no effects on mortality rates. Examples of these medications include cyclosporine, tacrolimus, methotrexate, budesonide, and metronidazole.

• #23 Primary sclerosing cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/diagnosis-treatment/drc-20355802

  Treatments for primary sclerosing cholangitis focus on managing complications and monitoring liver damage. Many medications have been studied in people with primary sclerosing cholangitis, but so far none have been found to slow or reverse the liver damage associated with this disease. […] Bile acid sequestrants. Medications that bind to bile acids the substances thought to cause itching in liver disease are the first line treatment for itching in primary sclerosing cholangitis. […] A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. […] A liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis. Though uncommon, it’s possible for primary sclerosing cholangitis to recur after a liver transplant.

• #24 Primary Sclerosing Cholangitis | PSC Disease Treatment – UChicago Medicine

  https://www.uchicagomedicine.org/conditions-services/liver-diseases-hepatology/psc-and-autoimmune-liver-disease/primary-sclerosing-cholangitis-psc

  Currently, there is no known treatment to cure or slow the progression of primary sclerosing cholangitis. Your healthcare provider, however, can treat the symptoms and complications associated with it, such as itching and bile duct infections. […] While there is no cure for primary sclerosing cholangitis, many of its complications can be managed. […] Medications such as bile acid sequestrants and antibiotics can help relieve the itching and infections caused by PSC. […] If primary sclerosing cholangitis eventually causes your liver to fail or leads to bile duct cancer, your doctor may recommend a liver transplant. Liver transplants can cure primary sclerosing cholangitis, although it may recur in the new liver in a small number of patients. […] Our liver disease specialists work closely with UChicago Medicine researchers to offer patients access to the advanced treatment options and clinical trials for primary sclerosing cholangitis.

• #25 Primary Sclerosing Cholangitis (PSC) — Irish Liver Foundation

  https://www.liverfoundation.ie/primary-sclerosing-cholangitis-psc

  Currently there is no known cure or specific treatment for PSC. Research is ongoing to find treatments that will improve patient outcomes in the future. Treatment to improve bile flow out of the liver, such as ursodeoxycholic acid (UDCA), or antibiotics such as vancomycin, may be trialled, but there is no definitive treatment at present. […] The main focus of care is to alleviate symptoms that can occur as a result of the disease process of PSC. Such treatments are listed below: […] Colestyramine (aka Questran) or Cholestagel/Colesevelam is a medication that is prescribed to help relieve itching. It works by reducing the uptake of bile acids in your system which can cause severe itching. […] When the flow of bile is significantly reduced, deficiencies of vitamin A, D, E, K can develop, and supplements may be prescribed.

• #26 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #27 Primary Sclerosing Cholangitis (PSC) – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/primary-sclerosing-cholangitis/

  At present there is no known cure or specific treatment for PSC. Clinical trials are happening to try to find treatments for the future. […] Symptoms such as itching can be treated with agents such as cholestyramine, rifampicin or naltrexone. […] Cholestyramine (trade name Questran or Questran Light) may be prescribed to help relieve itching. […] For a few people who eventually go on to get advanced cirrhosis, a liver transplant may be recommended when their quality of life has deteriorated and medical treatment can no longer control their symptoms. […] Endoscopic treatment of the bile ducts is aimed at reducing or halting the progression of the disease, usually by stretching or dilating the narrowed bile ducts. […] In later stages of the disease liver transplantation is often an option.

• #28 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #29 Primary Sclerosing Cholangitis (PSC)

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-sclerosing-cholangitis-psc/

  Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts. […] There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow. […] Over time, many PSC patients will continue to suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery. […] In addition, drug therapy trials, involving a large number of patients around the world, are exploring the potential use of several additional medications to lessen the symptoms and control liver damage.

• #30 Primary Sclerosing Cholangitis | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/primary-sclerosing-cholangitis

  Treatment for primary sclerosing cholangitis (PSC) includes medication to reduce itching and jaundice, antibiotics to treat infections and vitamin supplements since people with PSC are often deficient in vitamins A, D and K. In some cases, surgery to open major blockages in the common bile duct also is necessary. In addition, liver transplantation may be required and in some cases, can cure PSC.

• #31 Primary Sclerosing Cholangitis | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/primary-sclerosing-cholangitis

  Treatment for primary sclerosing cholangitis (PSC) includes medication to reduce itching and jaundice, antibiotics to treat infections and vitamin supplements since people with PSC are often deficient in vitamins A, D and K. In some cases, surgery to open major blockages in the common bile duct also is necessary. In addition, liver transplantation may be required and in some cases, can cure PSC.

• #32 Primary Sclerosing Cholangitis (PSC)

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-sclerosing-cholangitis-psc/

  Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts. […] There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow. […] Over time, many PSC patients will continue to suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery. […] In addition, drug therapy trials, involving a large number of patients around the world, are exploring the potential use of several additional medications to lessen the symptoms and control liver damage.

• #33 Primary sclerosing cholangitis | Altru Health System

  https://www.altru.org/health-library/conditions/primary-sclerosing-cholangitis

  Ursodeoxycholic acid (UDCA). Also known as ursodiol, UDCA is a naturally occurring bile acid that may help relieve itching symptoms caused by liver disease by increasing the absorbability of bile. […] Bile that backs up in narrowed or blocked ducts causes frequent bacterial infections. To prevent and treat these infections, people with primary sclerosing cholangitis may take repeated courses of antibiotics or continue taking antibiotics for long periods. […] Primary sclerosing cholangitis makes it difficult for your body to absorb certain vitamins. […] Your doctor may recommend vitamin supplements that you take as tablets or that you receive as an infusion through a vein in your arm. […] Blockages that occur in your bile ducts may be due to disease progression but can be a sign of cancer of the bile duct.

• #34 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #35 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #36 Primary Sclerosing Cholangitis (PSC)

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-sclerosing-cholangitis-psc/

  Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts. […] There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow. […] Over time, many PSC patients will continue to suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery. […] In addition, drug therapy trials, involving a large number of patients around the world, are exploring the potential use of several additional medications to lessen the symptoms and control liver damage.

• #37 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #38 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Currently, there is no treatment that can slow or stop primary sclerosing cholangitis from progressing. You can treat some of the symptoms and complications directly. For example, your healthcare provider may prescribe: […] Your healthcare provider will also keep your liver and bile ducts under surveillance. As the disease progresses, they may be able to periodically intervene by opening up a blocked bile duct. They can do this through an ERCP (endoscopic retrograde cholangiopancreatography), a type of exam that allows access to your bile ducts without surgery. […] This is a temporary solution, however. Over the course of 10 to 20 years, primary sclerosing cholangitis eventually progresses from early to late-stage liver disease, and finally, liver failure. Your healthcare provider will monitor your liver damage to determine when you should begin to consider liver transplantation. You’ll need to pass certain qualifications to get on a liver transplantation waiting list.

• #39 Primary sclerosing cholangitis | Altru Health System

  https://www.altru.org/health-library/conditions/primary-sclerosing-cholangitis

  Endoscopic retrograde cholangiopancreatography (ERCP) can help determine the cause, and bile duct blockage can be treated with: Balloon dilation. This procedure can open blockages in the larger bile ducts outside the liver. […] Stent placement. In this procedure, your doctor uses an endoscope and attached instruments to place a small plastic tube called a stent in a blocked bile duct to hold the duct open. […] A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. […] A liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis.

• #40 Primary Sclerosing Cholangitis Treatment | South Denver GI

  https://southdenvergi.com/conditions-procedures/bile-duct/primary-sclerosing-cholangitis/

  As this disease progresses, blockages may occur in the bile ducts. Bile duct blockages may be addressed through procedures such as balloon dilation or stent placement. […] In situations where the liver has failed, a liver transplant is an option. During this procedure, the surgeon will remove your diseased liver and replace it with a healthy donor liver.

• #41 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #42 Primary sclerosing cholangitis | Altru Health System

  https://www.altru.org/health-library/conditions/primary-sclerosing-cholangitis

  Endoscopic retrograde cholangiopancreatography (ERCP) can help determine the cause, and bile duct blockage can be treated with: Balloon dilation. This procedure can open blockages in the larger bile ducts outside the liver. […] Stent placement. In this procedure, your doctor uses an endoscope and attached instruments to place a small plastic tube called a stent in a blocked bile duct to hold the duct open. […] A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. […] A liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis.

• #43 Primary sclerosing cholangitis – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/primary-sclerosing-cholangitis/

  Management is primarily supportive and includes: […] Liver transplantation is the only curative treatment but has a significant risk of disease recurrence. […] Ursodeoxycholic acid (UDCA) is widely used, but evidence of benefit is mixed. […] Immunosuppressants (e.g., glucocorticoids) are usually reserved for patients with overlap syndromes. […] Perform interventional treatment as needed in patients with relevant strictures. […] Patients with PSC are at increased risk for post-ERCP cholangitis. […] Refer patients with decompensated cirrhosis and a MELD score 14. […] Patients with PSC are at increased risk of IBD, progression of liver disease to cirrhosis, osteoporosis, and malignancy.

• #44 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #45 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Management of primary sclerosing cholangitis (PSC) can be divided into endoscopic, medical, and surgical treatments. […] Ursodeoxycholic acid is the most promising disease-modifying agent. […] Corticosteroids and other immunosuppressive agents do not have a proven role. […] Cholestyramine and rifampicin improve pruritis but response to these agents cannot be reliably predicted. […] Opioid antagonists are useful in refractory pruritis. […] All patients with advanced PSC should be offered bone mineral-density measurement and, if needed, treatment for osteoporosis. […] Liver transplantation is the only therapy that improves survival. […] In 2006, PSC management can be divided into endoscopic, medical, and surgical treatments. […] Endoscopic therapy is primarily used to manage dominant strictures.

• #46 Management Of Primary Sclerosing Cholangitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5368615/

  Medical treatment is directed both at modifying the course of the disease and at symptomatic relief. […] Surgical therapy currently is limited to liver transplantation. […] Ursodeoxycholic acid (UDCA) is the most commonly used drug to treat PSC. […] UDCA has a choleretic effect that increases bile flow and thus excretion of potentially toxic bile acids and other metabolites. […] The only controlled trial compared prednisone, in combination with colchicine, to concurrent historical controls. […] Corticosteroids can be beneficial in patients who have been diagnosed as having a PSC/autoimmune hepatitis overlap syndrome. […] Liver transplantation is the only form of therapy that improves survival in PSC patients who develop end-stage liver disease. […] Treatment of PSC remains a work in progress. […] Among disease-modifying agents, UDCA is the most promising. […] Endoscopic therapy is the approach of choice for dominant strictures. […] For patients with end-stage liver disease due to PSC, liver transplantation is the only therapy that improves survival.

• #47 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #48 Primary sclerosing cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/diagnosis-treatment/drc-20355802

  Treatments for primary sclerosing cholangitis focus on managing complications and monitoring liver damage. Many medications have been studied in people with primary sclerosing cholangitis, but so far none have been found to slow or reverse the liver damage associated with this disease. […] Bile acid sequestrants. Medications that bind to bile acids the substances thought to cause itching in liver disease are the first line treatment for itching in primary sclerosing cholangitis. […] A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. […] A liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis. Though uncommon, it’s possible for primary sclerosing cholangitis to recur after a liver transplant.

• #49 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #50 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  In addition to liver transplantation, procedures include restorative proctocolectomy (RPC) with ileal pouch anal anastomosis (IPAA) for patients with ulcerative colitis and biliary reconstructive procedures. […] Posttransplantation complications include recurrence of PSC, worsening of inflammatory bowel disease (IBD) activity, and de novo IBD occurrence.

• #51 Primary Sclerosing Cholangitis (PSC) – Hepatic and Biliary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/primary-sclerosing-cholangitis-psc

  Asymptomatic patients usually require only monitoring (eg, physical examination and liver tests twice/year) and, if adults, periodic imaging and measurement of CA 19-9 for gallbladder cancer and cholangiocarcinoma screening. […] Liver transplantation is the only treatment that improves life expectancy in patients with primary sclerosing cholangitis and that offers a cure. […] Recurrent bacterial cholangitis, complications of end-stage liver disease (eg, intractable ascites, portosystemic encephalopathy, bleeding esophageal varices), or cholangiocarcinoma (in appropriately selected patients) are indications for liver transplantation. […] Supportive care […] Endoscopic retrograde cholangiopancreatography (ERCP) dilation for major (dominant) strictures […] Transplantation for recurrent bacterial cholangitis or complications of liver failure.

• #52 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #53 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #54 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #55 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #56 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #57 Primary Sclerosing Cholangitis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/27738

  Surgical options for PSC include biliary reconstructive procedures like choledochoduodenostomy, in which the surgeon attaches the common bile duct to the duodenum, and choledochojejunostomy, in which the surgeon attaches the common bile duct to the jejunum, and liver transplantation. Liver transplantation is the definitive treatment for patients with decompensated cirrhosis. Patients whose model for end-stage liver disease score exceeds 14 should be referred for liver transplantation. Orthotopic liver transplantation has 5-year survival rates of up to 80% in patients with PSC. […] General Measures: Bile acid sequestrants such as cholestyramine should be taken to reduce symptoms in patients with PSC and moderate pruritus. Rifampin and naltrexone are second-line treatment agents, which can be considered if cholestyramine is ineffective or poorly tolerated. Patients with PSC should undergo bone mineral density testing at the time of diagnosis and then every 2 to 4 years. Fat-soluble vitamin deficiencies are common in patients with advanced liver disease; clinicians should screen for and monitor patients for these. […] There is no definite cure for PSC at the time of this writing. Significant advances have been made in the early diagnosis of PSC, however, which is essential so that treatment can be started before developing liver failure.

• #58 Primary sclerosing cholangitis – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/primary-sclerosing-cholangitis/

  Management is primarily supportive and includes: […] Liver transplantation is the only curative treatment but has a significant risk of disease recurrence. […] Ursodeoxycholic acid (UDCA) is widely used, but evidence of benefit is mixed. […] Immunosuppressants (e.g., glucocorticoids) are usually reserved for patients with overlap syndromes. […] Perform interventional treatment as needed in patients with relevant strictures. […] Patients with PSC are at increased risk for post-ERCP cholangitis. […] Refer patients with decompensated cirrhosis and a MELD score 14. […] Patients with PSC are at increased risk of IBD, progression of liver disease to cirrhosis, osteoporosis, and malignancy.

• #59 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. Immunosuppressants, bile salts, chelators (eg, cholestyramine for pruritus), and steroids are used in an attempt to control the disease process but have not shown significant benefit. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. Suitability for liver transplantation should be determined expeditiously and, if appropriate, offered to the patient. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression.

• #60 Primary Sclerosing Cholangitis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/27738

  Surgical options for PSC include biliary reconstructive procedures like choledochoduodenostomy, in which the surgeon attaches the common bile duct to the duodenum, and choledochojejunostomy, in which the surgeon attaches the common bile duct to the jejunum, and liver transplantation. Liver transplantation is the definitive treatment for patients with decompensated cirrhosis. Patients whose model for end-stage liver disease score exceeds 14 should be referred for liver transplantation. Orthotopic liver transplantation has 5-year survival rates of up to 80% in patients with PSC. […] General Measures: Bile acid sequestrants such as cholestyramine should be taken to reduce symptoms in patients with PSC and moderate pruritus. Rifampin and naltrexone are second-line treatment agents, which can be considered if cholestyramine is ineffective or poorly tolerated. Patients with PSC should undergo bone mineral density testing at the time of diagnosis and then every 2 to 4 years. Fat-soluble vitamin deficiencies are common in patients with advanced liver disease; clinicians should screen for and monitor patients for these. […] There is no definite cure for PSC at the time of this writing. Significant advances have been made in the early diagnosis of PSC, however, which is essential so that treatment can be started before developing liver failure.

• #61 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797

  A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease may recur in the transplanted liver in a small number of patients. […] Care for primary sclerosing cholangitis focuses on monitoring liver function, managing symptoms and, when possible, doing procedures that temporarily open blocked bile ducts.

• #62 Primary sclerosing cholangitis – Wikipedia

  https://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis

  No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a liver transplant, but disease recurrence can occur in 25-30% of cases. For patients unable or unwilling to receive a transplant, therapy primarily focuses on relieving symptoms, rather than stopping disease progression. […] Supportive treatment for PSC symptoms is the cornerstone of management. These therapies are aimed at relieving symptoms such as itching with antipruritics (e.g. bile acid sequestrants such as cholestyramine); antibiotics to treat episodes of ascending cholangitis; and vitamin supplements, as people with PSC are often deficient in fat-soluble vitamins (A, D, E, and K). […] Liver transplantation is the only proven long-term treatment of PSC. Indications for transplantation include recurrent bacterial ascending cholangitis, decompensated cirrhosis, hepatocellular carcinoma, hilar cholangiocarcinoma, and complications of portal hypertension. Not all patients are candidates for liver transplantation, and some experience disease recurrence afterward.

• #63 Primary Sclerosing Cholangitis, Part 1: Epidemiology, Etiopathogenesis, Clinical Features, and Treatment – Gastroenterology & Hepatology

  https://www.gastroenterologyandhepatology.net/archives/may-2018/primary-sclerosing-cholangitis-part-1-epidemiology-etiopathogenesis-clinical-features-and-treatment/

  Recurrent PSC (rPSC) is an enduring clinical dilemma. It occurs in 1.8% to 36.8% of LT recipients and is associated with a greater need for repeat LT, a 4-fold increased risk of death, and decreased overall survival compared to patients who remain rPSC-free. […] Overall, there continues to be progress in the understanding and management of this disease, with potential on the horizon.

• #64 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  While UDCA (15 mg/kg/day) is considered the first-line treatment for PBC, its effectiveness in PSC patients is less clear. A randomized, placebo-controlled trial involving 105 PSC patients who received 13 to 15 mg/kg/day of UDCA or a placebo showed a significant reduction in ALP levels, although there were no significant effects on histological progression or the time to liver transplantation. […] The effects of higher doses of UDCA (28 to 30 mg/kg/day) were assessed in a long-term, 5-year, randomized, double-blind, controlled trial. Although this higher dosage significantly lowered serum ALP and ALT levels, the study concluded that serious adverse events were more common in the UDCA than placebo group. […] NorUDCA has demonstrated anti-inflammatory, anti-fibrotic, and anti-proliferative effects in preclinical models of PSC. It has also been shown to lower serum levels of ALP, γGT, AST, and ALT in phase 2 trials involving PBC patients, with a phase 3 trial currently underway to assess its impact on histological progression. […] OCA has been approved as a second-line add-on therapy to UDCA in PBC due to evidence of biochemical efficacy in a phase III study. In PSC patients, 5–10 mg OCA once a day for 24 weeks was found to be partially effective in reducing ALP levels, with no change in fibrosis markers.

• #65

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  Nevertheless, opinions differ on the role of UDCA in the treatment of PSC, mainly due to the fact that despite a marked improvement of serum markers of cholestasis, no significant improvement of transplant-free survival was found in UDCA-treated patients. […] In moderate doses (15-20 mg/kg) UDCA may exert protective effects in the hepatobiliary tract, but its effectiveness as monotherapy is probably not sufficient to prevent PSC progression in a majority of patients. […] Nevertheless, discontinuation of UDCA has been shown to cause worsening of symptoms, of serum liver tests and of the Mayo Risk Score and should therefore be well justified in patients stable on therapy. […] 24-Norursodeoxycholic acid (nor-UDCA) is a side chain-shortened UDCA homologue that is conjugated only at very limited rates and can be passively absorbed by cholangiocytes and undergo cholehepatic shunting with the induction of bicarbonate-rich choleresis again strengthening the biliary bicarbonate umbrella, but anti-inflammatory, anti-lipotoxic, anti-fibrotic and anti-proliferative effects were also proposed.

• #66

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  In a phase II clinical trial, nor-UDCA dose-dependently reduced serum ALP, GGT, AST, and ALT levels. […] A multicenter phase III trial is now ongoing with a focus on endpoints such as histological progression and change in ALP levels. […] Obeticholic acid (OCA), a semi-synthetic 6-ethyl analogue of chenodeoxycholic acid, acts as a potent FXR agonist affecting bile acid synthesis, inflammation, and liver fibrosis. […] In PSC, a phase II clinical trial demonstrated reduced ALP levels in the group receiving a 5-10 mg dose of OCA, but no change in fibrosis markers. […] Vancomycin is now evaluated in phase III. […] In some patients, fecal transplantation may also be an interesting treatment option as reported recently.

• #67 New hope for primary sclerosing cholangitis treatment | MDedge

  https://mdedge.com/gihepnews/article/108174/hepatology/new-hope-primary-sclerosing-cholangitis-treatment

  New hope for primary sclerosing cholangitis treatment […] A modified form of ursodeoxycholic acid (UDCA) could offer patients with primary sclerosing cholangitis the first real pharmacologic treatment option, it was reported at the International Liver Congress. […] Phase II study findings showed that a 1,500-mg daily dose of norursodeoxycholic acid (norUDCA) significantly (P less than .0001) reduced the primary endpoint of serum alkaline phosphatase (ALP) by 26% versus baseline levels within 12 weeks of treatment. […] While this investigational drug is still only a symptomatic therapy and not a cure, it brings a viable option for managing the devastating but rare liver disease that currently lacks any effective therapy other than liver transplantation. […] At the moment there is no medical treatment for PSC, […] We are very excited about these data because it is a new hope for this type of patient. […] The fact that we have nothing to offer at the moment that works as a medical treatment makes this study so particular. […] norUDCA reduced ALP in a dose-dependent fashion.

• #68

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  In a phase II clinical trial, nor-UDCA dose-dependently reduced serum ALP, GGT, AST, and ALT levels. […] A multicenter phase III trial is now ongoing with a focus on endpoints such as histological progression and change in ALP levels. […] Obeticholic acid (OCA), a semi-synthetic 6-ethyl analogue of chenodeoxycholic acid, acts as a potent FXR agonist affecting bile acid synthesis, inflammation, and liver fibrosis. […] In PSC, a phase II clinical trial demonstrated reduced ALP levels in the group receiving a 5-10 mg dose of OCA, but no change in fibrosis markers. […] Vancomycin is now evaluated in phase III. […] In some patients, fecal transplantation may also be an interesting treatment option as reported recently.

• #69

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  In a phase II clinical trial, nor-UDCA dose-dependently reduced serum ALP, GGT, AST, and ALT levels. […] A multicenter phase III trial is now ongoing with a focus on endpoints such as histological progression and change in ALP levels. […] Obeticholic acid (OCA), a semi-synthetic 6-ethyl analogue of chenodeoxycholic acid, acts as a potent FXR agonist affecting bile acid synthesis, inflammation, and liver fibrosis. […] In PSC, a phase II clinical trial demonstrated reduced ALP levels in the group receiving a 5-10 mg dose of OCA, but no change in fibrosis markers. […] Vancomycin is now evaluated in phase III. […] In some patients, fecal transplantation may also be an interesting treatment option as reported recently.

• #70 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  Cilofexor, a nonsteroidal FXR agonist, has been also tested in phase 2 trials in PBC patients, while a phase III clinical trial, the PRIMIS study, has been discontinued. […] The future role of FXR agonists in PSC therapy may hinge on the use of combination therapies that target multiple mechanisms of action, given the complexity and multifactorial nature of the disease. […] FGF-19 plays a crucial role in inhibiting bile acid synthesis by downregulating CYP7A1 after binding to its receptor, the FGFR4. […] The oncogenic potential of FGF19 analogue should be carefully weighted, especially in patients with PSC, who are at increased risk of developing cholangiocarcinoma. […] Targeting the gut microbiota as a therapeutic strategy for PSC presents a compelling area for exploration. […] Multiple antibiotics, including metronidazole, azithromycin, vancomycin, and minocycline, have been investigated as therapies for PSC, with some showing promising effects in small studies. The most widely investigated antibiotic is vancomycin.

• #71 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  Cilofexor, a nonsteroidal FXR agonist, has been also tested in phase 2 trials in PBC patients, while a phase III clinical trial, the PRIMIS study, has been discontinued. […] The future role of FXR agonists in PSC therapy may hinge on the use of combination therapies that target multiple mechanisms of action, given the complexity and multifactorial nature of the disease. […] FGF-19 plays a crucial role in inhibiting bile acid synthesis by downregulating CYP7A1 after binding to its receptor, the FGFR4. […] The oncogenic potential of FGF19 analogue should be carefully weighted, especially in patients with PSC, who are at increased risk of developing cholangiocarcinoma. […] Targeting the gut microbiota as a therapeutic strategy for PSC presents a compelling area for exploration. […] Multiple antibiotics, including metronidazole, azithromycin, vancomycin, and minocycline, have been investigated as therapies for PSC, with some showing promising effects in small studies. The most widely investigated antibiotic is vancomycin.

• #72 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  Cilofexor, a nonsteroidal FXR agonist, has been also tested in phase 2 trials in PBC patients, while a phase III clinical trial, the PRIMIS study, has been discontinued. […] The future role of FXR agonists in PSC therapy may hinge on the use of combination therapies that target multiple mechanisms of action, given the complexity and multifactorial nature of the disease. […] FGF-19 plays a crucial role in inhibiting bile acid synthesis by downregulating CYP7A1 after binding to its receptor, the FGFR4. […] The oncogenic potential of FGF19 analogue should be carefully weighted, especially in patients with PSC, who are at increased risk of developing cholangiocarcinoma. […] Targeting the gut microbiota as a therapeutic strategy for PSC presents a compelling area for exploration. […] Multiple antibiotics, including metronidazole, azithromycin, vancomycin, and minocycline, have been investigated as therapies for PSC, with some showing promising effects in small studies. The most widely investigated antibiotic is vancomycin.

• #73

  https://journals.lww.com/ajg/fulltext/2020/06000/targeting_the_gut_microbiome_as_a_treatment_for.9.aspx

  The fact that an antibiotic improved outcomes may further suggest that microbes are involved in the pathophysiology of PSC. […] Treatment with vancomycin specifically targets Gram-positive bacteria, which includes various Clostridium spp. known to be primarily involved with the dehydroxylation of primary BAs, into the secondary BAs present in the distal small intestine and colon. […] Fecal microbiota transplantation (FMT) refers to the transfer of intestinal microbes from a healthy donor into a recipient with the intent of modifying the recipient’s intestinal microbiome. […] Larger controlled studies are needed to define efficacy and define the mechanisms of FMT in PSC.

• #74

  https://journals.lww.com/ajg/fulltext/2020/06000/targeting_the_gut_microbiome_as_a_treatment_for.9.aspx

  The fact that an antibiotic improved outcomes may further suggest that microbes are involved in the pathophysiology of PSC. […] Treatment with vancomycin specifically targets Gram-positive bacteria, which includes various Clostridium spp. known to be primarily involved with the dehydroxylation of primary BAs, into the secondary BAs present in the distal small intestine and colon. […] Fecal microbiota transplantation (FMT) refers to the transfer of intestinal microbes from a healthy donor into a recipient with the intent of modifying the recipient’s intestinal microbiome. […] Larger controlled studies are needed to define efficacy and define the mechanisms of FMT in PSC.

• #75 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  To date, one study has reported the effects of FMT in PSC. This was an open-label pilot study carried out in 10 patients with PSC with concurrent IBD and ALP 1.5× the upper limit of normal. The results demonstrated that FMT in PSC is safe. In addition, increases in bacterial diversity and engraftment were found to correlate with an improvement in ALP. […] Despite much progress, therapy for PSC remains suboptimal, as is our understanding of its pathophysiology and the development of effective medical treatments. At present, liver transplantation remains the sole curative treatment for patients with end-stage liver disease or those experiencing severe complications such as intractable pruritus or recurrent cholangitis. Current data show that patients receiving deceased donor allografts have high 5-year survival rates of ≈85%.

• #76

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  Results of the most recently published randomized, placebo-controlled FITCH trial (fibrates for cholestasis-associated itch) clearly showed the efficacy of bezafibrate (400 mg/day) in the treatment of severe or moderate cholestasis-associated pruritus in PSC and PBC. […] Thus, bezafibrate may become the 1st line medical treatment of pruritus in PSC in the future. […] Ursodeoxycholic acid (UDCA), a hydrophilic human bile acid, represents the first-line therapy in the treatment of PBC (13-15 mg/kg/day) where it leads to improved transplant-free survival in all patients under study. […] In PSC and other very rare chronic cholestatic diseases, much less data are available on long-term treatment with UDCA, but due to its potent anticholestatic effects and its excellent safety profile when administered at moderate doses of 13-20 mg/kg/day, UDCA is widely prescribed at least in Continental Europe where the so far best LTx-free survival data for patients with PSC have been reported.

• #77 Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments

  https://www.mdpi.com/2073-4409/13/19/1650

  Primary sclerosing cholangitis (PSC) is a rare, chronic liver disease with no approved therapies. The ursodeoxycholic acid (UDCA) has been widely used, although there is no evidence that the use of UDCA delays the time to liver transplant or increases survival. Several candidate drugs are currently being developed. The largest group of these new agents is represented by FXR agonists, including obeticholic acid, cilofexor, and tropifexor. Other agents that target bile acid metabolism are ASTB/IBAP inhibitors and fibroblasts growth factor (FGF)19 analogues. […] Additional agents under evaluation are PPARs (elafibranor and seladelpar), anti-itching agents such as MAS-related G-protein–coupled receptors antagonists, and anti-fibrotic and immunosuppressive agents. Drugs targeting gut bacteria and bile acid pathways are also under investigation, given the strong link between PSC and gut microbiota.

• #78 Novel Therapies for Primary Sclerosing Cholangitis – Practical Gastro

  https://practicalgastro.com/2018/11/30/novel-therapies-for-primary-sclerosing-cholangitis/

  A clinical trial evaluating the safety and efficacy of simtuzumab (SIM, a humanized IgG4 monoclonal antibody against LOXL2) in PSC was conducted. […] Results from a phase 2 clinical trial evaluating the safety and efficacy of norursodeoxycholic in patients with PSC were presented at the EASL ILC in 2016; 59 PSC patients were randomized to 500, 1000, and 1500 mg of norursodeoxycholic for 12 weeks. […] Interruption of intestinal bile acid circulation might have therapeutic benefit in PSC. […] Cenicriviroc, a dual chemokine receptor (CCR) 5 and CCR2 antagonist is currently being studied for PSC. […] NGM282 is a variant of the human hormone FGF19 that reduces liver fat content, reverses fibrosis and improves liver function. […] No therapy thus far has been effective to slow the progression of disease and complications from cirrhosis in PSC.

• #79 Novel Therapies for Primary Sclerosing Cholangitis – Practical Gastro

  https://practicalgastro.com/2018/11/30/novel-therapies-for-primary-sclerosing-cholangitis/

  A clinical trial evaluating the safety and efficacy of simtuzumab (SIM, a humanized IgG4 monoclonal antibody against LOXL2) in PSC was conducted. […] Results from a phase 2 clinical trial evaluating the safety and efficacy of norursodeoxycholic in patients with PSC were presented at the EASL ILC in 2016; 59 PSC patients were randomized to 500, 1000, and 1500 mg of norursodeoxycholic for 12 weeks. […] Interruption of intestinal bile acid circulation might have therapeutic benefit in PSC. […] Cenicriviroc, a dual chemokine receptor (CCR) 5 and CCR2 antagonist is currently being studied for PSC. […] NGM282 is a variant of the human hormone FGF19 that reduces liver fat content, reverses fibrosis and improves liver function. […] No therapy thus far has been effective to slow the progression of disease and complications from cirrhosis in PSC.

• #80 Novel Therapies for Primary Sclerosing Cholangitis – Practical Gastro

  https://practicalgastro.com/2018/11/30/novel-therapies-for-primary-sclerosing-cholangitis/

  A clinical trial evaluating the safety and efficacy of simtuzumab (SIM, a humanized IgG4 monoclonal antibody against LOXL2) in PSC was conducted. […] Results from a phase 2 clinical trial evaluating the safety and efficacy of norursodeoxycholic in patients with PSC were presented at the EASL ILC in 2016; 59 PSC patients were randomized to 500, 1000, and 1500 mg of norursodeoxycholic for 12 weeks. […] Interruption of intestinal bile acid circulation might have therapeutic benefit in PSC. […] Cenicriviroc, a dual chemokine receptor (CCR) 5 and CCR2 antagonist is currently being studied for PSC. […] NGM282 is a variant of the human hormone FGF19 that reduces liver fat content, reverses fibrosis and improves liver function. […] No therapy thus far has been effective to slow the progression of disease and complications from cirrhosis in PSC.

• #81 Stem Cell Treatment for Primary Sclerosing Cholangitis ( PSC )

  https://stemcellthailand.org/therapies/primary-sclerosing-cholangitis-psc/

  Recent advancements in stem cell therapy have emerged as a promising investigational treatment for Primary Sclerosing Cholangitis (PSC), targeting both inflammatory processes and biliary regeneration. […] Stem cell therapy represents a novel therapeutic frontier for managing Primary Sclerosing Cholangitis, offering potential immunomodulatory and regenerative benefits. While preliminary data suggests promise in reducing biliary inflammation and hepatic fibrosis, rigorous clinical trials are essential to establish long-term safety profiles and standardized protocols. […] Clinical trials have shown promising results in reducing inflammation and improving liver function markers. However, ideal, suitable, or favorable timing, dosing, and delivery methods remain under investigation. Combining stem cell therapy with conventional treatments may enhance therapeutic outcomes for PSC patients. However, more long-term controlled studies are needed to establish clinical efficacy and consistent results.

• #82

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10-15% cholangiocarcinoma, and ultimately end-stage liver disease. […] Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. […] Still, several new drugs are under investigation for combined medical treatment representing the potential to improve survival and clinical outcomes in PSC. […] The so-far recommended first-line medical treatment is cholestyramine (4-16 g/day, administered separately from other drugs). […] In case of its ineffectiveness or intolerance, rifampicin, naltrexone, and sertraline may be considered as the following steps.

• #83

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  Results of the most recently published randomized, placebo-controlled FITCH trial (fibrates for cholestasis-associated itch) clearly showed the efficacy of bezafibrate (400 mg/day) in the treatment of severe or moderate cholestasis-associated pruritus in PSC and PBC. […] Thus, bezafibrate may become the 1st line medical treatment of pruritus in PSC in the future. […] Ursodeoxycholic acid (UDCA), a hydrophilic human bile acid, represents the first-line therapy in the treatment of PBC (13-15 mg/kg/day) where it leads to improved transplant-free survival in all patients under study. […] In PSC and other very rare chronic cholestatic diseases, much less data are available on long-term treatment with UDCA, but due to its potent anticholestatic effects and its excellent safety profile when administered at moderate doses of 13-20 mg/kg/day, UDCA is widely prescribed at least in Continental Europe where the so far best LTx-free survival data for patients with PSC have been reported.

• #84 Primary sclerosing cholangitis | Altru Health System

  https://www.altru.org/health-library/conditions/primary-sclerosing-cholangitis

  Ursodeoxycholic acid (UDCA). Also known as ursodiol, UDCA is a naturally occurring bile acid that may help relieve itching symptoms caused by liver disease by increasing the absorbability of bile. […] Bile that backs up in narrowed or blocked ducts causes frequent bacterial infections. To prevent and treat these infections, people with primary sclerosing cholangitis may take repeated courses of antibiotics or continue taking antibiotics for long periods. […] Primary sclerosing cholangitis makes it difficult for your body to absorb certain vitamins. […] Your doctor may recommend vitamin supplements that you take as tablets or that you receive as an infusion through a vein in your arm. […] Blockages that occur in your bile ducts may be due to disease progression but can be a sign of cancer of the bile duct.

• #85 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797

  A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease may recur in the transplanted liver in a small number of patients. […] Care for primary sclerosing cholangitis focuses on monitoring liver function, managing symptoms and, when possible, doing procedures that temporarily open blocked bile ducts.

• #86 Primary Sclerosing Cholangitis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/27738

  Surgical options for PSC include biliary reconstructive procedures like choledochoduodenostomy, in which the surgeon attaches the common bile duct to the duodenum, and choledochojejunostomy, in which the surgeon attaches the common bile duct to the jejunum, and liver transplantation. Liver transplantation is the definitive treatment for patients with decompensated cirrhosis. Patients whose model for end-stage liver disease score exceeds 14 should be referred for liver transplantation. Orthotopic liver transplantation has 5-year survival rates of up to 80% in patients with PSC. […] General Measures: Bile acid sequestrants such as cholestyramine should be taken to reduce symptoms in patients with PSC and moderate pruritus. Rifampin and naltrexone are second-line treatment agents, which can be considered if cholestyramine is ineffective or poorly tolerated. Patients with PSC should undergo bone mineral density testing at the time of diagnosis and then every 2 to 4 years. Fat-soluble vitamin deficiencies are common in patients with advanced liver disease; clinicians should screen for and monitor patients for these. […] There is no definite cure for PSC at the time of this writing. Significant advances have been made in the early diagnosis of PSC, however, which is essential so that treatment can be started before developing liver failure.

• #87 Primary Sclerosing Cholangitis (PSC) – Hepatic and Biliary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/primary-sclerosing-cholangitis-psc

  Asymptomatic patients usually require only monitoring (eg, physical examination and liver tests twice/year) and, if adults, periodic imaging and measurement of CA 19-9 for gallbladder cancer and cholangiocarcinoma screening. […] Liver transplantation is the only treatment that improves life expectancy in patients with primary sclerosing cholangitis and that offers a cure. […] Recurrent bacterial cholangitis, complications of end-stage liver disease (eg, intractable ascites, portosystemic encephalopathy, bleeding esophageal varices), or cholangiocarcinoma (in appropriately selected patients) are indications for liver transplantation. […] Supportive care […] Endoscopic retrograde cholangiopancreatography (ERCP) dilation for major (dominant) strictures […] Transplantation for recurrent bacterial cholangitis or complications of liver failure.

• #88 Primary Sclerosing Cholangitis (PSC) — Irish Liver Foundation

  https://www.liverfoundation.ie/primary-sclerosing-cholangitis-psc

  Endoscopic dilatation or stretching of the bile ducts is an option for some patients. The placement of a stent inside the bile duct to prevent the narrowing from progressing is also an option for some patients with narrowing in a main bile duct. […] Liver transplant is the ultimate treatment for PSC patients who go on to develop advanced liver scarring, also known as cirrhosis. […] Patient with advanced PSC are also at risk of developing bile duct cancer. […] Colitis, causing diarrhoea, is common in PSC, so a screening colonoscopy will be organised to investigate for this. […] If IBD is present, a colonoscopy is performed annually to screen and detect bowel cancer at an early stage, as there is an increased risk in patients with PSC and IBD. […] Osteoporosis is associated with PSC. This is diagnosed by a bone density scan called a DEXA scan. If detected, medication such as vitamin D, calcium replacement etc can be prescribed to protect your bones, and to reduce the risk of development of fragility fractures.

• #89 Treatment for Primary Sclerosing Cholangitis – NIDDK

  https://www.niddk.nih.gov/health-information/liver-disease/primary-sclerosing-cholangitis/treatment

  Currently, no cure or effective treatments for primary sclerosing cholangitis (PSC) exist. However, doctors can treat narrowed or blocked bile ducts and symptoms of PSC. […] If bile ducts are narrowed or blocked, doctors may use endoscopic retrograde cholangiopancreatography (ERCP) to open them and help keep them open. To help keep ducts open, doctors sometimes place stents. Stents are tiny tubes that a doctor leaves in narrowed ducts for a short time to hold them open. […] Doctors may recommend treatments for liver complications of PSC. […] If PSC leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, medical procedures, or surgery. […] If cirrhosis leads to liver failure, you may need a liver transplant. […] Doctors may consider a liver transplant if PSC leads to liver failure or causes severe symptoms or complications. In some patients with bile duct cancer, liver transplant may be the best treatment. Doctors consider liver transplants only after all other treatment options have failed. […] If you have PSC, you can take steps to help prevent further liver damage.

• #90 Primary Sclerosing Cholangitis | Diagnosis and Treatment

  https://patient.info/digestive-health/primary-sclerosing-cholangitis

  Liver transplantation is the only treatment able to modify the outlook of the disease. It may be considered if your liver is affected badly. The outlook following a liver transplant is good. However, PSC recurs in around a fifth of cases following a liver transplant. […] Most people with PSC will be advised to eat a normal healthy diet. Ideally, anybody with inflammation of the liver should not drink alcohol, or only in very small amounts. If you already have liver inflammation, alcohol may increase the risk and speed of developing cirrhosis – where normal liver tissue is replaced by scar tissue (fibrosis).

• #91 Primary Sclerosing Cholangitis (PSC): Diagnosis and Treatment

  https://www.healthline.com/health/psc-liver

  Lifestyle changes: You may need to reduce or avoid your alcohol intake and quit smoking to further protect your liver. Eating a balanced, nutritious diet and taking supplements may also help address nutritional deficiencies. […] Vaccination: Your doctor may recommend getting vaccinated against both hepatitis A and B to further protect your liver. […] A liver transplant may be necessary later stages of PSC. […] Experts say liver transplants are effective, but its possible for PSC to come back even with a transplant. […] The outlook for people who have undergone a liver transplant for PSC is good. Around 80% survive beyond 2 years after surgery. […] Many people with PSC may need a liver transplant around 10 years after their diagnosis. The good news is that after a transplant, you may go on to live a healthy, full life.

• #92 Primary Sclerosing Cholangitis (PSC): Diagnosis and Treatment

  https://www.healthline.com/health/psc-liver

  Lifestyle changes: You may need to reduce or avoid your alcohol intake and quit smoking to further protect your liver. Eating a balanced, nutritious diet and taking supplements may also help address nutritional deficiencies. […] Vaccination: Your doctor may recommend getting vaccinated against both hepatitis A and B to further protect your liver. […] A liver transplant may be necessary later stages of PSC. […] Experts say liver transplants are effective, but its possible for PSC to come back even with a transplant. […] The outlook for people who have undergone a liver transplant for PSC is good. Around 80% survive beyond 2 years after surgery. […] Many people with PSC may need a liver transplant around 10 years after their diagnosis. The good news is that after a transplant, you may go on to live a healthy, full life.

• #93 Primary sclerosing cholangitis: Symptoms, causes, diagnosis

  https://www.medicalnewstoday.com/articles/190952

  Treatment includes ursodiol, or ursodeoxycholic acid, a bile acid naturally produced by the liver. […] Research shows that it can lower elevated liver enzyme levels in people with PSC. However, it does not improve the liver’s condition or overall survival rates. […] A doctor may also prescribe: medications to relieve itching, called antipruritics; bile acid sequestrants, such as cholestyramine, to reduce cholesterol; antibiotics to treat infections; vitamin supplements. […] If a person also has ulcerative colitis, the doctor will recommend appropriate treatment. […] If fluid retention from cirrhosis results in swelling in the abdomen and feet, a low-salt diet and diuretics may help. […] Researchers have tried using various immunosuppressants and antibiotics to manage PSC, but these have had no effects on mortality rates. Examples of these medications include cyclosporine, tacrolimus, methotrexate, budesonide, and metronidazole.

• #94 Primary Sclerosing Cholangitis (PSC): Diagnosis and Treatment

  https://www.healthline.com/health/psc-liver

  Lifestyle changes: You may need to reduce or avoid your alcohol intake and quit smoking to further protect your liver. Eating a balanced, nutritious diet and taking supplements may also help address nutritional deficiencies. […] Vaccination: Your doctor may recommend getting vaccinated against both hepatitis A and B to further protect your liver. […] A liver transplant may be necessary later stages of PSC. […] Experts say liver transplants are effective, but its possible for PSC to come back even with a transplant. […] The outlook for people who have undergone a liver transplant for PSC is good. Around 80% survive beyond 2 years after surgery. […] Many people with PSC may need a liver transplant around 10 years after their diagnosis. The good news is that after a transplant, you may go on to live a healthy, full life.

• #95 Novel Therapies for Primary Sclerosing Cholangitis – Practical Gastro

  https://practicalgastro.com/2018/11/30/novel-therapies-for-primary-sclerosing-cholangitis/

  Primary sclerosing cholangitis (PSC) is chronic, cholestatic liver disease that progresses to advanced liver disease and cirrhosis. In this article we present new studies, clinical trials and novel therapies for the treatment PSC. […] Currently there is no medical therapy that will halt the progression of liver disease in PSC patients, despite numerous clinical trials over the past two decades. This is due to uncertainty regarding the pathophysiology of PSC and also lack of reliable diagnostic markers. […] Multiple clinical trials have studied the efficacy and safety of ursodeoxycholic acid (UDCA) in PSC patients. UDCA is the most commonly prescribed drug for PSC, as it is effective in other cholestatic liver diseases, specifically primary biliary cholangitis (PBC). However, the role of UDCA in clinical improvement is questionable.

• #96 Novel Therapies for Primary Sclerosing Cholangitis – Practical Gastro

  https://practicalgastro.com/2018/11/30/novel-therapies-for-primary-sclerosing-cholangitis/

  Primary sclerosing cholangitis (PSC) is chronic, cholestatic liver disease that progresses to advanced liver disease and cirrhosis. In this article we present new studies, clinical trials and novel therapies for the treatment PSC. […] Currently there is no medical therapy that will halt the progression of liver disease in PSC patients, despite numerous clinical trials over the past two decades. This is due to uncertainty regarding the pathophysiology of PSC and also lack of reliable diagnostic markers. […] Multiple clinical trials have studied the efficacy and safety of ursodeoxycholic acid (UDCA) in PSC patients. UDCA is the most commonly prescribed drug for PSC, as it is effective in other cholestatic liver diseases, specifically primary biliary cholangitis (PBC). However, the role of UDCA in clinical improvement is questionable.

• #97 Primary Sclerosing Cholangitis Treatment Varies Significantly Among Care Centers

  https://www.ajmc.com/view/primary-sclerosing-cholangitis-treatment-varies-significantly-among-care-centers

  A new survey of European health care centers with expertise in managing primary sclerosing cholangitis (PSC) suggests there is considerable intercenter variation in treatment and surveillance practices. […] The authors of the report, published in JHEP Reports, say their data show there needs to be better harmonization of PSC treatment strategies. […] Ursodeoxycholic acid (UDCA) is often used to treat PSC, but the investigators said its use is controversial. Immunosuppressive drugs and antibiotics are also sometimes prescribed, they said. […] When it came to UDCA, half of physicians said they always prescribe the therapy to people with PSC, but 12% said they never do. […] Sixty-five percent of physicians continued UDCA treatment regardless of treatment response, while 23% of physicians discontinued UDCA if no biochemical or clinical response was observed, the authors said.

• #98 Primary Sclerosing Cholangitis Treatment Varies Significantly Among Care Centers

  https://www.ajmc.com/view/primary-sclerosing-cholangitis-treatment-varies-significantly-among-care-centers

  A minority of physicians used other therapies, including vancomycin and immunosuppressives in select cases (12% of physicians and 37% of physicians, respectively). […] Taken together, the investigators said their survey responses show a lack of uniformity in PSC care in Europe. […] Overall, we found apparent uncertainties and discrepancies between practice and published guidelines and our results confirm that existing data and recommendations for clinicians are inadequate for uniform patient management, as shown by the overt heterogeneity in responses, they said. […] They added it will be challenging to align treatment practices more closely given the current variance in strategies, but they said the goal is important. […] Already established international networks and research associations should work to coordinate guideline recommendations in order to generate a better basis for developing clinical trials and to improve the daily management of patients with PSC, they wrote.

• #99 Primary Sclerosing Cholangitis | Children’s Liver Disease Foundation

  https://childliverdisease.org/liver-information/childhood-liver-conditions/primary-sclerosing-cholangitis/

  The slow progressive nature of PSC has made clinical trials more difficult but new treatments are constantly under investigation. There are multi-centre clinical trials for paediatric patients taking place internationally to find answers. Furthermore, there are several areas of active investigation in this field with promising clinical data from adults with PSC. Personalised medicine and treatments tailored to individual patients may be the future of PSC therapy.

• #100 Primary Sclerosing Cholangitis Treatment Varies Significantly Among Care Centers

  https://www.ajmc.com/view/primary-sclerosing-cholangitis-treatment-varies-significantly-among-care-centers

  A new survey of European health care centers with expertise in managing primary sclerosing cholangitis (PSC) suggests there is considerable intercenter variation in treatment and surveillance practices. […] The authors of the report, published in JHEP Reports, say their data show there needs to be better harmonization of PSC treatment strategies. […] Ursodeoxycholic acid (UDCA) is often used to treat PSC, but the investigators said its use is controversial. Immunosuppressive drugs and antibiotics are also sometimes prescribed, they said. […] When it came to UDCA, half of physicians said they always prescribe the therapy to people with PSC, but 12% said they never do. […] Sixty-five percent of physicians continued UDCA treatment regardless of treatment response, while 23% of physicians discontinued UDCA if no biochemical or clinical response was observed, the authors said.

• #101 Primary Sclerosing Cholangitis Treatment Varies Significantly Among Care Centers

  https://www.ajmc.com/view/primary-sclerosing-cholangitis-treatment-varies-significantly-among-care-centers

  A minority of physicians used other therapies, including vancomycin and immunosuppressives in select cases (12% of physicians and 37% of physicians, respectively). […] Taken together, the investigators said their survey responses show a lack of uniformity in PSC care in Europe. […] Overall, we found apparent uncertainties and discrepancies between practice and published guidelines and our results confirm that existing data and recommendations for clinicians are inadequate for uniform patient management, as shown by the overt heterogeneity in responses, they said. […] They added it will be challenging to align treatment practices more closely given the current variance in strategies, but they said the goal is important. […] Already established international networks and research associations should work to coordinate guideline recommendations in order to generate a better basis for developing clinical trials and to improve the daily management of patients with PSC, they wrote.

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