Materiały źródłowe

• #1 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  There’s no cure for primary biliary cholangitis, but medicines are available to help slow the progression of the disease and prevent complications. Options include: […] Ursodeoxycholic acid. This medicine, also known as UDCA or ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn’t cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. […] Obeticholic acid (Ocaliva). Studies show that when obeticholic acid is given alone or combined with ursodiol for 12 months, it can help improve liver function and slow liver fibrosis. […] Fibrates (Tricor). Researchers aren’t exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. […] Budesonide. When combined with UDCA, the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis.

• #2 Primary Biliary Cholangitis: What It Is, Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/17715-primary-biliary-cholangitis-pbc

  Primary biliary cholangitis is a chronic and progressive condition that causes inflammation and, eventually, the destruction of the bile ducts that run through your liver. Without working bile ducts, bile backs up in your liver, causing liver damage. This can lead to cirrhosis of the liver. But medication can delay and sometimes prevent it. […] Theres no cure for PBC, but you can slow it down and improve your condition with medication. Ursodeoxycholic acid (UDCA) is a type of bile salt that can help clear bile from your liver and reduce liver damage. It works well for about half of people with PBC, especially in the early stages. For those who dont benefit from UDCA, doctors sometimes recommend a different bile salt called obeticholic acid. […] If medication doesnt improve your condition and your liver function continues to decline, your doctor may put you on the liver transplant waiting list. Liver transplant surgery has excellent results for people with PBC. Although, like other autoimmune diseases, PBC may return after your liver transplant, but it tends to progress much more slowly the second time around. Life expectancy after your transplant is normal.

• #2 Primary Biliary Cholangitis: Symptoms & Causes

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-biliary-cholangitis-pbc/

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic liver disease resulting from progressive destruction of the bile ducts in the liver called the intrahepatic bile ducts. […] There is no cure for Primary Biliary Cholangitis (PBC), but there is medication available to manage the disease and slow down the progression of liver damage. […] There are medications that can help slow disease progression and manage symptoms. Ursodiol (brand names Actigall, URSO 250, URSO Forte) is a naturally occurring bile acid (ursodeoxycholic acid or UDCA) that helps move bile out of the liver and into the small intestine. […] If used early enough, Ursodiol can improve liver function and may keep you from needing, or delay the need for a liver transplant. […] In May 2016, obeticholic acid (brand name Ocaliva) was approved for the treatment of Primary Biliary Cholangitis (PBC) in combination with UDCA in adults with an inadequate response to UDCA, or as a single therapy in adults unable to tolerate UDCA.

• #3 Primary Biliary Cirrhosis | Conditions and Treatments | Center for Liver Disease & Transplantation | Columbia University Department of Surgery

  https://columbiasurgery.org/conditions-and-treatments/primary-biliary-cirrhosis

  Primary biliary cirrhosis (also called primary biliary cholangitis) is a chronic disease that slowly damages and destroys the bile ducts. This causes bile to build up in the liver, which can lead to progressive scarring and cirrhosis. […] While there is no cure, various treatments are available that can slow the progression of the disease, relieve symptoms, and prevent complications. […] There is no cure for primary biliary cirrhosis. However, there are treatment options that can help reduce and manage its complications. The following are some of the most common treatments: […] A variety of medications are available that can help improve liver function, reduce scarring and inflammation, move bile through the liver, and treat other symptoms. Ursodeoxycholic acid (also known as ursodiol) is commonly used first. […] In severe cases, a liver transplantation may be necessary. This involves surgically removing the diseased liver and replacing it with a healthy donor liver. In some cases, primary biliary cirrhosis can recur in the new liver.

• #3 Primary biliary cholangitis – Wikipedia

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

  Medical therapy of PBC primarily targets disease progression and symptom control. The first-line treatment for PBC is ursodeoxycholic acid (UDCA). UDCA has been the only drug available for two decades and more recently obeticholic acid (OCA), a semi-synthetic hydrophobic bile acid analogue, has been licensed as a second-line option for patients with inadequate UDCA response or who are intolerant to UDCA. Several other agents have been studied, including immunosuppressants, but robust evidence of benefit is lacking. […] UDCA improves liver enzyme levels, slows down histological progression, and improves liver transplant-free survival. UDCA also reduces the need for liver transplantation. UDCA should be taken at a dose of 13 to 15 mg per kg of body weight per day, usually in two divided doses each day. Liver chemistries usually improve within a few weeks of starting UDCA, and 90% of any benefit is observed after 6-9 months of therapy. Liver chemistries should be re-evaluated after 1 year of treatment. UDCA is usually continued lifelong. Up to 40% of people do not respond to treatment with UDCA. Patients with PBC who have an inadequate response to UDCA or those few (less than 3%) who are intolerant to UDCA are candidates for second-line therapies.

• #4

  https://link.springer.com/article/10.1007/s12072-021-10276-6

  Primary biliary cholangitis (PBC) is a chronic intrahepatic cholestatic disease with not fully elucidated pathogenesis. […] Currently, ursodeoxycholic acid (UDCA) is the treatment of choice for this disease. […] UDCA is the treatment of choice and most established therapy for PBC patients, recommended by major national and international clinical practice guidelines. […] It is recommended that oral UDCA (13~15 mg/kg/day) should be standard therapy for all PBC patients. […] Unfortunately, about 30-40% of PBC patients show insufficient biochemical responses to UDCA and remain at risk for disease progression to advanced stages, including cirrhosis. […] There is no consensus on therapies for patients with insufficient biochemical response to UDCA. […] OCA has been approved as second-line therapy for PBC in the United States and Europe.

• #5 Current Landscape and Evolving Therapies for Primary Biliary Cholangitis

  https://www.mdpi.com/2073-4409/13/18/1580

  Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disorder characterized by progressive cholestatic that, if untreated, can progress to liver fibrosis, cirrhosis and liver decompensation requiring liver transplant. […] Thus, while bile acid-based therapies, specifically ursodeoxycholic acid and obeticholic acid, have been the cornerstone of therapy in PBC, novel therapeutic approaches have been developed in recent years. […] The introduction of ursodeoxycholic acid (UDCA), a secondary bile acid normally present in human bile at low concentrations, in the treatment of PBC patients has changed the disease course, improving transplant-free survival rates to up to 60% of patients, thereby reducing mortality. Currently, life expectancy in PBC patients under UDCA therapy is similar to non-PBC patients.

• #6 Primary biliary cholangitis: diagnosis and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/primary-biliary-cholangitis-diagnosis-and-management

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a progressive autoimmune cholestatic liver disease that results in end-stage liver disease and the need for liver transplantation if left untreated. […] Treatment of PBC aims to slow disease progression and reduce the need for transplantation. Even with treatment, PBC remains a progressive disease with risk of liver-related complications and death. […] The first-line treatment for PBC is ursodeoxycholic acid (UDCA). UDCA is an endogenous bile acid that exerts anti-inflammatory, immunomodulatory and anti-apoptotic effects within the liver through bile enrichment and displacement of toxic hydrophobic bile acids. […] UDCA is recommended at a dose of 13–15mg/kg/day, continued life-long. […] A licensed second-line oral therapy is obeticholic acid (OCA), which modulates bile acid synthesis, and increases transport of bile acids out of hepatocytes, limiting hepatic exposure.

• #7 Primary Biliary Cirrhosis: New Options | Borland Groover

  https://borlandgroover.com/patient-resources/gastroenterology/gi-education/primary-biliary-cirrhosis-new-options

  Primary Biliary Cirrhosis is always treated, even in the early, asymptomatic stages. Ursodeoxycholate (UDCA, Urso, ursodiol, Actigall) at a dose of 13-15 mg/kg/day (generally 1000-1500 mg daily) is approved for PBC by the U.S. Food and Drug Administration. Studies have demonstrated consistent evidence of biochemical improvement, histologic improvement and in some cases improved survival with the use of UDCA. UDCA use has resulted in a decreased need for liver transplantation. Therapy is life-long as acceleration of disease has occurred when UDCA is withheld. The drug has rare side effects; mild diarrhea, weight gain or thinning of the hair sometimes occurs. One clinical caveat is that different UDCA preparations do not have equal bioavailability and, therefore, this may be one condition for which generic drug substitution is unwise. It is also important that UDCA be taken at least two hours prior to or after bile acid binding agents such as cholestyramine or colestid.

• #8 Primary biliary cholangitis: Promising emerging therapies | HMER

  https://www.dovepress.com/primary-biliary-cholangitis-promising-emerging-innovative-therapies-an-peer-reviewed-fulltext-article-HMER

  A cohort study including 3902 patients with PBC found that treatment with UDCA prolonged liver transplant-free survival; irrespective of the stage of the disease, UDCA significantly reduced the risk of LT or death. […] The GLOBE score was introduced in 2015 by the GLOBE-PBC Study Group. […] This score estimates the risk of liver transplantation or mortality after a year of UDCA therapy. […] The open-label extension of this trial revealed that the results were durable after six years of follow-up. […] A post hoc analysis by Harms et al in the POISE study reported that 12 months of OCA treatment resulted in the reduction of APRI and GLOBE scores. […] Their study suggested that addition of bezafibrate to UDCA improves the long-term prognosis in patients with PBC. […] A recent study by Hansen et al reported an improvement in the GLOBE score after two years of treatment with seladelpar.

• #9 Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/171117-treatment

  The goals of treatment of primary biliary cholangitis (PBC) are to slow the progression rate of the disease and to alleviate the symptoms (eg, pruritus, osteoporosis, sicca syndrome). Liver transplantation appears to be the only life-saving procedure. […] UDCA is the major medication used to slow the progression of the disease. Patients with early disease have clinical, biochemical, and histologic improvement with UDCA treatment. Reports suggest that UDCA delays the need for transplantation and delays death. The efficacy of this medication in late stages (ie, cirrhosis) is questionable. Patients who achieve biochemical response to UDCA after 1 year of treatment reportedly have a similar survival rate to the matched control population, and this observation might be used to identify the population of nonresponders who will require alternative or additional treatments.

• #10 Treating primary biliary cholangitis – British Liver Trust

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

  As soon as you are diagnosed with primary biliary cholangitis (PBC), your specialist will prescribe treatment. The aim of your treatment is to: […] Treatment is life long, but try not to be alarmed by this. PBC is generally very slow to develop and the treatment should slow it down further. […] If the first treatment you take doesnt help, there are alternatives your doctor will suggest. […] If your PBC cant be controlled with medicine, then a liver transplant is also an option. […] This is most likely to be the first treatment your doctor suggests. […] Urso comes as a capsule that you swallow. […] Your doctor will use regular blood tests to check how well Urso is working. […] If blood tests show that Urso isnt working well enough for you, your doctor may suggest adding another medicine called obeticholic acid (sometimes called OCA).

• #11 Management of Primary Biliary Cholangitis: Current Treatment and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10081121/

  Primary biliary cholangitis is an autoimmune cholestatic liver disease characterized by progressive destruction of bile ducts, which can ultimately progress to chronic liver disease and cirrhosis. Ursodeoxycholic acid and obeticholic acid are the only 2 Food and Drug Administration (FDA)-approved medications for primary biliary cholangitis. […] Unfortunately, up to 40% of patients with primary biliary cholangitis have an incomplete response to ursodeoxycholic acid, warranting an essential need for additional therapeutics. […] First-line treatment for primary biliary cholangitis (PBC) includes initiation of ursodeoxycholic acid (UDCA). Obeticholic acid (OCA) should be added if there is a sub-optimal response to UDCA, but OCA is contraindicated in patients with decompensated cirrhosis and compensated cirrhosis with evidence of portal hypertension.

• #12 Management of Primary Biliary Cholangitis: Current Treatment and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10081121/

  Up to 40% of patients with PBC have an incomplete response to UDCA, this patient population requires additional pharmacotherapies with the goal of inducing biochemical remission. […] Peroxisome proliferator-activated receptor agonists including bezafibrate, fenofibrate, and elafibranor have shown promise in inducing biochemical remission in PBC patients who are UDCA non-responders. […] Additional investigational therapies including fibroblast growth factor-19 agonists, farnesoid X receptor agonists, and NADPH oxidase 3 inhibitors are being explored as potential disease-modifying therapies. […] Therefore, additional therapeutics for the management of PBC, particularly for those with advanced fibrosis and UDCA non-responders, are urgently needed. […] Obeticholic acid has been shown to be effective in UDCA non-responders and currently is considered second-line therapy. However, it is contraindicated in cirrhotic patients who have evidence of portal hypertension. […] There is a great need for additional pharmacotherapy to induce disease remission in PBC patients who do not respond to UDCA. […] Fibroblast growth factor-19 agonists and NOX inhibitors are also under investigation for further DMT options.

• #13 Primary Biliary Cholangitis: Symptoms & Causes

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-biliary-cholangitis-pbc/

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic liver disease resulting from progressive destruction of the bile ducts in the liver called the intrahepatic bile ducts. […] There is no cure for Primary Biliary Cholangitis (PBC), but there is medication available to manage the disease and slow down the progression of liver damage. […] There are medications that can help slow disease progression and manage symptoms. Ursodiol (brand names Actigall, URSO 250, URSO Forte) is a naturally occurring bile acid (ursodeoxycholic acid or UDCA) that helps move bile out of the liver and into the small intestine. […] If used early enough, Ursodiol can improve liver function and may keep you from needing, or delay the need for a liver transplant. […] In May 2016, obeticholic acid (brand name Ocaliva) was approved for the treatment of Primary Biliary Cholangitis (PBC) in combination with UDCA in adults with an inadequate response to UDCA, or as a single therapy in adults unable to tolerate UDCA.

• #14 5 New Supportive Therapies for Primary Biliary Cholangitis | myPBCteam

  https://www.mypbcteam.com/resources/5-new-supportive-therapies-for-primary-biliary-cholangitis

  Many liver disease treatments address the liver itself but dont quite treat your symptoms. Primary biliary cholangitis (PBC) supportive therapies help manage uncomfortable problems like itchy skin and dry mouth and eyes. But how do you know which treatments are right for you? […] Doctors typically treat PBC with medications that help slow disease progression. The U.S. Food and Drug Administration has approved four treatments for PBC. They include: Ursodeoxycholic acid (UDCA or Ursodiol), Elafibranor (Iqirvo), Seladelpar (Livdelzi), Obeticholic acid (Ocaliva). […] While treatments like UDCA and obeticholic acid help treat the underlying cause of PBC, they dont always help manage symptoms. Your doctor may recommend supportive therapies to help make you more comfortable with PBC. These treatments support your other PBC medications and improve your overall quality of life.

• #15 Primary biliary cholangitis: diagnosis and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/primary-biliary-cholangitis-diagnosis-and-management

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a progressive autoimmune cholestatic liver disease that results in end-stage liver disease and the need for liver transplantation if left untreated. […] Treatment of PBC aims to slow disease progression and reduce the need for transplantation. Even with treatment, PBC remains a progressive disease with risk of liver-related complications and death. […] The first-line treatment for PBC is ursodeoxycholic acid (UDCA). UDCA is an endogenous bile acid that exerts anti-inflammatory, immunomodulatory and anti-apoptotic effects within the liver through bile enrichment and displacement of toxic hydrophobic bile acids. […] UDCA is recommended at a dose of 13–15mg/kg/day, continued life-long. […] A licensed second-line oral therapy is obeticholic acid (OCA), which modulates bile acid synthesis, and increases transport of bile acids out of hepatocytes, limiting hepatic exposure.

• #16 Update on Emerging Treatment Options for Primary Biliary Cholangitis | HMER

  https://www.dovepress.com/update-on-emerging-treatment-options-for-primary-biliary-cholangitis-peer-reviewed-fulltext-article-HMER

  The widespread use of ursodeoxycholic acid (UDCA) since it was approved in 1997 by the United States Food and Drug Administration has dramatically changed the natural disease course of PBC, including decreased progression to liver transplantation (LT) in this patient population. […] Standard of care for PBC includes treatment with 1315 mg/kg/day of UDCA in divided doses. […] UDCA has also been studied for use in recurrent PBR (rPBC) after LT. […] OCA, a farnesoid X receptor (FXR) agonist, is a more potent analogue of CDCA. OCA facilitates bile acid homeostasis by suppressing de novo bile acid synthesis and increasing choleresis. […] Fibrates are peroxisome proliferator activator receptor (PPAR) agonists. […] Bezafibrate has been used in Japan for over a decade as a second-line agent in PBC.

• #17 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  There’s no cure for primary biliary cholangitis, but medicines are available to help slow the progression of the disease and prevent complications. Options include: […] Ursodeoxycholic acid. This medicine, also known as UDCA or ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn’t cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. […] Obeticholic acid (Ocaliva). Studies show that when obeticholic acid is given alone or combined with ursodiol for 12 months, it can help improve liver function and slow liver fibrosis. […] Fibrates (Tricor). Researchers aren’t exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. […] Budesonide. When combined with UDCA, the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis.

• #18 Primary biliary cholangitis – Wikipedia

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

  The initial treatment for PBC is almost always ursodeoxycholic acid, but some patients may need alternative or additional medications for managing their cholestasis. For these patients, options include obeticholic acid, the PPAR agonists elafibranor and seladelpar, and the off-label use of fibrates. […] Obeticholic acid (OCA) is FDA-approved for the treatment of PBC in individuals intolerant or unresponsive to UDCA. OCA is a farnesoid X receptor agonist, and results in increased bile flow (choleresis). OCA is started at 5 mg daily, and liver chemistries should be rechecked after 3 months of treatment. If the liver chemistries remain elevated, then the dose of OCA may be increased to 10 mg per day. The most common side effect of OCA is pruritus. […] Fibric acid derivatives, or fibrates, are agonists of the peroxisome proliferator activator receptor (PPAR), a nuclear receptor involved in several metabolic pathways. While fibrates are approved for the treatment of hypertriglyceridemia, they exert anticholestatic effects and have been studied for the treatment of PBC and are used off-label as a second-line option for patients who do not respond sufficiently to UDCA. Among the fibrates, bezafibrate and fenofibrate, PPAR-alpha selective agonists, have been extensively studied as therapeutic agents because of their potential ability to decrease bile acid synthesis and bile acid-related hepatic inflammation. A randomized, controlled trial in 2018 showed its efficacy in patients with inadequate response to UDCA. While fibrates can be considered as off-label treatment for PBC that does not respond to UDCA, they should not be used in decompensated cirrhosis.

• #19 Management of Primary Biliary Cholangitis: Current Treatment and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10081121/

  Primary biliary cholangitis is an autoimmune cholestatic liver disease characterized by progressive destruction of bile ducts, which can ultimately progress to chronic liver disease and cirrhosis. Ursodeoxycholic acid and obeticholic acid are the only 2 Food and Drug Administration (FDA)-approved medications for primary biliary cholangitis. […] Unfortunately, up to 40% of patients with primary biliary cholangitis have an incomplete response to ursodeoxycholic acid, warranting an essential need for additional therapeutics. […] First-line treatment for primary biliary cholangitis (PBC) includes initiation of ursodeoxycholic acid (UDCA). Obeticholic acid (OCA) should be added if there is a sub-optimal response to UDCA, but OCA is contraindicated in patients with decompensated cirrhosis and compensated cirrhosis with evidence of portal hypertension.

• #20 Primary biliary cholangitis – Wikipedia

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

  The initial treatment for PBC is almost always ursodeoxycholic acid, but some patients may need alternative or additional medications for managing their cholestasis. For these patients, options include obeticholic acid, the PPAR agonists elafibranor and seladelpar, and the off-label use of fibrates. […] Obeticholic acid (OCA) is FDA-approved for the treatment of PBC in individuals intolerant or unresponsive to UDCA. OCA is a farnesoid X receptor agonist, and results in increased bile flow (choleresis). OCA is started at 5 mg daily, and liver chemistries should be rechecked after 3 months of treatment. If the liver chemistries remain elevated, then the dose of OCA may be increased to 10 mg per day. The most common side effect of OCA is pruritus. […] Fibric acid derivatives, or fibrates, are agonists of the peroxisome proliferator activator receptor (PPAR), a nuclear receptor involved in several metabolic pathways. While fibrates are approved for the treatment of hypertriglyceridemia, they exert anticholestatic effects and have been studied for the treatment of PBC and are used off-label as a second-line option for patients who do not respond sufficiently to UDCA. Among the fibrates, bezafibrate and fenofibrate, PPAR-alpha selective agonists, have been extensively studied as therapeutic agents because of their potential ability to decrease bile acid synthesis and bile acid-related hepatic inflammation. A randomized, controlled trial in 2018 showed its efficacy in patients with inadequate response to UDCA. While fibrates can be considered as off-label treatment for PBC that does not respond to UDCA, they should not be used in decompensated cirrhosis.

• #21

  https://link.springer.com/article/10.1007/s12072-021-10276-6

  Obeticholic acid was generally well tolerated after long-term follow-up, with pruritus (77%) and fatigue (33%) being the most common adverse events in phase III POISE trial. […] It is recommended that bezafibrate (400 mg/day) or fenofibrate (200 mg/day) could be added to UDCA for patients with an inadequate response to UDCA monotherapy. […] Budesonide (69 mg/day) might be added to non-cirrhotic PBC patients with suboptimal response to UDCA. […] Pruritus is one of the characteristic symptoms of cholestasis and results in impaired health-related HRQoL in PBC patients. […] To date, cholestyramine is the first-line therapy for pruritus caused by cholestasis. […] It is recommended that cholestyramine (4-16 g/day) as the first-line therapy for pruritus. […] The mechanisms for metabolic bone diseases (bone loss and osteoporosis) in PBC patients are complicated, involving the defect of absorption of fat-soluble vitamins and the direct effect of cholestasis on bone metabolism.

• #22 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  There’s no cure for primary biliary cholangitis, but medicines are available to help slow the progression of the disease and prevent complications. Options include: […] Ursodeoxycholic acid. This medicine, also known as UDCA or ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn’t cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. […] Obeticholic acid (Ocaliva). Studies show that when obeticholic acid is given alone or combined with ursodiol for 12 months, it can help improve liver function and slow liver fibrosis. […] Fibrates (Tricor). Researchers aren’t exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. […] Budesonide. When combined with UDCA, the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis.

• #23 Primary biliary cholangitis: diagnosis and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/primary-biliary-cholangitis-diagnosis-and-management

  Fibrates (e.g. bezafibrate) are an unlicensed treatment for PBC that may be considered in those unresponsive or intolerant to OCA. […] Off-label budesonide has also been used in PBC, with trials using doses from 6–9mg orally per day. […] Patients who meet minimal listing criteria using the United Kingdom Model for End-Stage Liver Disease (UKELD) score may be considered for liver transplantation.

• #24 Primary biliary cholangitis – Wikipedia

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

  The initial treatment for PBC is almost always ursodeoxycholic acid, but some patients may need alternative or additional medications for managing their cholestasis. For these patients, options include obeticholic acid, the PPAR agonists elafibranor and seladelpar, and the off-label use of fibrates. […] Obeticholic acid (OCA) is FDA-approved for the treatment of PBC in individuals intolerant or unresponsive to UDCA. OCA is a farnesoid X receptor agonist, and results in increased bile flow (choleresis). OCA is started at 5 mg daily, and liver chemistries should be rechecked after 3 months of treatment. If the liver chemistries remain elevated, then the dose of OCA may be increased to 10 mg per day. The most common side effect of OCA is pruritus. […] Fibric acid derivatives, or fibrates, are agonists of the peroxisome proliferator activator receptor (PPAR), a nuclear receptor involved in several metabolic pathways. While fibrates are approved for the treatment of hypertriglyceridemia, they exert anticholestatic effects and have been studied for the treatment of PBC and are used off-label as a second-line option for patients who do not respond sufficiently to UDCA. Among the fibrates, bezafibrate and fenofibrate, PPAR-alpha selective agonists, have been extensively studied as therapeutic agents because of their potential ability to decrease bile acid synthesis and bile acid-related hepatic inflammation. A randomized, controlled trial in 2018 showed its efficacy in patients with inadequate response to UDCA. While fibrates can be considered as off-label treatment for PBC that does not respond to UDCA, they should not be used in decompensated cirrhosis.

• #25 Primary Biliary Cholangitis: Symptoms & Causes

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-biliary-cholangitis-pbc/

  Liver transplantation is considered when medical treatment no longer sufficiently controls the disease. […] Other alternative therapies in patients who are incomplete responders to UDCA include fenofibrate. Medications to suppress the immune system may also be prescribed including prednisone or azathioprine in Primary Biliary Cholangitis (PBC) patients with the overlap syndrome with autoimmune hepatitis. […] Intense itching is one of the most common symptoms of Primary Biliary Cholangitis (PBC). […] Blood tests to monitor for deficiencies in fat-soluble vitamins are often done. […] As Primary Biliary Cholangitis (PBC) progresses, some people need to replace the fat-soluble vitamins lost in fatty stools, so you may be put on vitamin A, D, E and K replacement therapy. […] Since people with Primary Biliary Cholangitis (PBC) are at a higher risk for osteoporosis, calcium and vitamin D are usually prescribed. […] As the ability of the liver to function decreases over time, complications associated with cirrhosis will need to be addressed and treated.

• #26 Treatment of primary biliary cholangitis-Industry News-Cascade Pharmaceuticals, Inc

  http://www.cascadepharm.com/en/NewsUpdates/157.html

  There are mainly two kinds of fibrates used in the second-line treatment of PBC, namely fenofibrate and bezafibrate. […] The combined treatment of immunosuppressant UDCA and immunosuppressant is mainly used in patients with PBC-AIH overlap syndrome. […] OCA is a derivative of chenodeoxycholic acid, which was approved for second-line treatment of clinical patients in May 2017. […] TUDCA is a binding bile acid formed by the carboxyl group of ursodeoxycholic acid and the amino dehydration of taurine. It has strong hydrophilicity, can reduce the toxicity of bile acids and protect hepatocytes from apoptosis, and has been approved by FDA for the treatment of primary biliary cholangitis. […] Traditional Chinese medicine theory holds that the main syndrome type of PBC is liver depression and spleen deficiency. […] Rituximab is an anti-CD20 monoclonal antibody, which can selectively eliminate B cells to treat autoimmune mediated liver diseases. […] MSc is a potential method to treat PBC. […] Some PBC patients need some adjuvant treatment to deal with their extrahepatic manifestations.

• #27 5 New Supportive Therapies for Primary Biliary Cholangitis | myPBCteam

  https://www.mypbcteam.com/resources/5-new-supportive-therapies-for-primary-biliary-cholangitis

  Many liver disease treatments address the liver itself but dont quite treat your symptoms. Primary biliary cholangitis (PBC) supportive therapies help manage uncomfortable problems like itchy skin and dry mouth and eyes. But how do you know which treatments are right for you? […] Doctors typically treat PBC with medications that help slow disease progression. The U.S. Food and Drug Administration has approved four treatments for PBC. They include: Ursodeoxycholic acid (UDCA or Ursodiol), Elafibranor (Iqirvo), Seladelpar (Livdelzi), Obeticholic acid (Ocaliva). […] While treatments like UDCA and obeticholic acid help treat the underlying cause of PBC, they dont always help manage symptoms. Your doctor may recommend supportive therapies to help make you more comfortable with PBC. These treatments support your other PBC medications and improve your overall quality of life.

• #28 Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Treatment & Management: Medical Care, Surgical Care

  https://emedicine.medscape.com/article/171117-treatment

  In May 2016, the FDA approved obeticholic acid to be used in combination with UDCA for PBC in adult patients with an inadequate response to UDCA, or as a single therapy in adults unable to tolerate UDCA. […] Elafibranor is indicated for PBC in combination with UDCA in adults who have had an inadequate response to UDCA, or as monotherapy in patients unable to tolerate UDCA. […] In August 2024, the FDA approved seladelpar for PBC in combination with UDCA in adults who have an inadequate response to UDCA, as well as monotherapy for PBC for patients are unable to tolerate UDCA. […] As the disease progresses to cirrhosis, an elevated bilirubin level, a prolonged prothrombin time, and a decreased albumin level can be found. The increased bilirubin level is an ominous sign of disease progression, and liver transplantation must be considered. It can also be considered for those with severe, refractory pruritus. Liver transplantation appears to be the only life-saving procedure, with excellent survival (upwards of 80% at 5 years).

• #29 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  There’s no cure for primary biliary cholangitis, but medicines are available to help slow the progression of the disease and prevent complications. Options include: […] Ursodeoxycholic acid. This medicine, also known as UDCA or ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn’t cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. […] Obeticholic acid (Ocaliva). Studies show that when obeticholic acid is given alone or combined with ursodiol for 12 months, it can help improve liver function and slow liver fibrosis. […] Fibrates (Tricor). Researchers aren’t exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. […] Budesonide. When combined with UDCA, the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis.

• #30

  https://link.springer.com/article/10.1007/s12072-021-10276-6

  Obeticholic acid was generally well tolerated after long-term follow-up, with pruritus (77%) and fatigue (33%) being the most common adverse events in phase III POISE trial. […] It is recommended that bezafibrate (400 mg/day) or fenofibrate (200 mg/day) could be added to UDCA for patients with an inadequate response to UDCA monotherapy. […] Budesonide (69 mg/day) might be added to non-cirrhotic PBC patients with suboptimal response to UDCA. […] Pruritus is one of the characteristic symptoms of cholestasis and results in impaired health-related HRQoL in PBC patients. […] To date, cholestyramine is the first-line therapy for pruritus caused by cholestasis. […] It is recommended that cholestyramine (4-16 g/day) as the first-line therapy for pruritus. […] The mechanisms for metabolic bone diseases (bone loss and osteoporosis) in PBC patients are complicated, involving the defect of absorption of fat-soluble vitamins and the direct effect of cholestasis on bone metabolism.

• #31 Primary biliary cholangitis: diagnosis and management – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/primary-biliary-cholangitis-diagnosis-and-management

  Fibrates (e.g. bezafibrate) are an unlicensed treatment for PBC that may be considered in those unresponsive or intolerant to OCA. […] Off-label budesonide has also been used in PBC, with trials using doses from 6–9mg orally per day. […] Patients who meet minimal listing criteria using the United Kingdom Model for End-Stage Liver Disease (UKELD) score may be considered for liver transplantation.

• #32 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] Antihistamines are commonly used to reduce itching. […] Cholestyramine is a powder that may stop itching. […] Rifampin is an antibiotic that may stop itching. […] Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. […] Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce itching.

• #33 Primary biliary cholangitis – Wikipedia

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

  Pruritus is a common symptom in people with PBC. First-line treatment of pruritus consists of anion-exchange resins, such as cholestyramine, colestipol, or colesevalam. These anion-exchange resins are nonabsorbed, highly positively charged substances that bind bile acids, which are negatively charged anions. Anion-exchange resins relieve itching caused by excess bile acids in circulation by binding bile acids in the gut and facilitating elimination. Bloating or constipation may occur with anion-exchange resins. Cholestyramine may affect absorption of UDCA; if cholestyramine is necessary, it should be taken at least 60 minutes before or 4 hours after UDCA is taken. […] Treatment options for pruritus that does not improve with anion-exchange resins include: rifampicin, naltrexone, or sertraline. Rifampicin may rarely cause drug induced liver injury and should be avoided if serum bilirubin is elevated (greater than 2.5 mg/dL). Liver enzymes should be monitored after starting rifampin. Opioid antagonists may cause a self-limited opioid withdrawal like reaction, with abdominal pain, elevated blood pressure, tachycardia, goose bumps, nightmares, and depersonalization. To avoid such reactions, the dose should start low and gradually be increased. […] In patients with advanced liver disease, the only curative therapy is liver transplant. Outcomes are favourable, with five-year patient survival rates better than for most other indications for LT (80-85%).

• #34 Primary biliary cholangitis – Wikipedia

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

  Pruritus is a common symptom in people with PBC. First-line treatment of pruritus consists of anion-exchange resins, such as cholestyramine, colestipol, or colesevalam. These anion-exchange resins are nonabsorbed, highly positively charged substances that bind bile acids, which are negatively charged anions. Anion-exchange resins relieve itching caused by excess bile acids in circulation by binding bile acids in the gut and facilitating elimination. Bloating or constipation may occur with anion-exchange resins. Cholestyramine may affect absorption of UDCA; if cholestyramine is necessary, it should be taken at least 60 minutes before or 4 hours after UDCA is taken. […] Treatment options for pruritus that does not improve with anion-exchange resins include: rifampicin, naltrexone, or sertraline. Rifampicin may rarely cause drug induced liver injury and should be avoided if serum bilirubin is elevated (greater than 2.5 mg/dL). Liver enzymes should be monitored after starting rifampin. Opioid antagonists may cause a self-limited opioid withdrawal like reaction, with abdominal pain, elevated blood pressure, tachycardia, goose bumps, nightmares, and depersonalization. To avoid such reactions, the dose should start low and gradually be increased. […] In patients with advanced liver disease, the only curative therapy is liver transplant. Outcomes are favourable, with five-year patient survival rates better than for most other indications for LT (80-85%).

• #35 Primary Biliary Cirrhosis: New Options | Borland Groover

  https://borlandgroover.com/patient-resources/gastroenterology/gi-education/primary-biliary-cirrhosis-new-options

  Important supportive treatments are available for the complications of PBC. For fatigue, patients should be assessed for anemia, hypothyroidism, depression and sleep disorders. Modafinil at a dose of 100-200 mg/day has been associated with decreased daytime somnolence. In addition to UDCA, pruritus may be treated with bile acid binding agents such as cholestyramine at 4-16 grams daily. Rifampin at a dose of 150 mg daily to twice daily has been shown to relieve pruritus. Opioid receptor antagonists including naloxone infused IV and naltrexone orally have been shown by meta-analysis to significantly reduce pruritus. Naltrexone is often started at a low dose such as 12.5 mg daily with a slow titration over several weeks to 50 mg daily to reduce the chance of producing an opioid withdrawal reaction.

• #36 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] Antihistamines are commonly used to reduce itching. […] Cholestyramine is a powder that may stop itching. […] Rifampin is an antibiotic that may stop itching. […] Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. […] Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce itching.

• #37 Treating primary biliary cholangitis – British Liver Trust

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

  OCA is a tablet you take once a day. […] Elafibranor was approved by NICE for patients in England and Wales in November 2024 and by the SMC for patients in Scotland in April 2025. […] This medicine is not yet specifically licensed for treating PBC, but doctors do commonly give it to patients. […] If Urso hasnt controlled your PBC as well as your doctor would like, adding bezafibrate can often help. […] PBC can cause itching and extreme tiredness (fatigue). […] If you have itching, your doctor will suggest a medicine called colestyramine. […] If you cant take colestyramine or it isnt helping, your doctor may suggest other medicines for itching. […] Unfortunately, there are no medicines your doctor can suggest that can help with fatigue.

• #38 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] Antihistamines are commonly used to reduce itching. […] Cholestyramine is a powder that may stop itching. […] Rifampin is an antibiotic that may stop itching. […] Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. […] Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce itching.

• #39 Primary Biliary Cirrhosis: New Options | Borland Groover

  https://borlandgroover.com/patient-resources/gastroenterology/gi-education/primary-biliary-cirrhosis-new-options

  Important supportive treatments are available for the complications of PBC. For fatigue, patients should be assessed for anemia, hypothyroidism, depression and sleep disorders. Modafinil at a dose of 100-200 mg/day has been associated with decreased daytime somnolence. In addition to UDCA, pruritus may be treated with bile acid binding agents such as cholestyramine at 4-16 grams daily. Rifampin at a dose of 150 mg daily to twice daily has been shown to relieve pruritus. Opioid receptor antagonists including naloxone infused IV and naltrexone orally have been shown by meta-analysis to significantly reduce pruritus. Naltrexone is often started at a low dose such as 12.5 mg daily with a slow titration over several weeks to 50 mg daily to reduce the chance of producing an opioid withdrawal reaction.

• #40 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] Antihistamines are commonly used to reduce itching. […] Cholestyramine is a powder that may stop itching. […] Rifampin is an antibiotic that may stop itching. […] Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. […] Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce itching.

• #41 Primary biliary cholangitis – Wikipedia

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

  Pruritus is a common symptom in people with PBC. First-line treatment of pruritus consists of anion-exchange resins, such as cholestyramine, colestipol, or colesevalam. These anion-exchange resins are nonabsorbed, highly positively charged substances that bind bile acids, which are negatively charged anions. Anion-exchange resins relieve itching caused by excess bile acids in circulation by binding bile acids in the gut and facilitating elimination. Bloating or constipation may occur with anion-exchange resins. Cholestyramine may affect absorption of UDCA; if cholestyramine is necessary, it should be taken at least 60 minutes before or 4 hours after UDCA is taken. […] Treatment options for pruritus that does not improve with anion-exchange resins include: rifampicin, naltrexone, or sertraline. Rifampicin may rarely cause drug induced liver injury and should be avoided if serum bilirubin is elevated (greater than 2.5 mg/dL). Liver enzymes should be monitored after starting rifampin. Opioid antagonists may cause a self-limited opioid withdrawal like reaction, with abdominal pain, elevated blood pressure, tachycardia, goose bumps, nightmares, and depersonalization. To avoid such reactions, the dose should start low and gradually be increased. […] In patients with advanced liver disease, the only curative therapy is liver transplant. Outcomes are favourable, with five-year patient survival rates better than for most other indications for LT (80-85%).

• #42 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] Antihistamines are commonly used to reduce itching. […] Cholestyramine is a powder that may stop itching. […] Rifampin is an antibiotic that may stop itching. […] Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. […] Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce itching.

• #43 5 New Supportive Therapies for Primary Biliary Cholangitis | myPBCteam

  https://www.mypbcteam.com/resources/5-new-supportive-therapies-for-primary-biliary-cholangitis

  People with PBC can take antihistamines to treat their itchy skin. […] Your doctor may also recommend cholestyramine to relieve your itchy skin. This medication helps your body clear out bile acids that can cause itchiness. […] Your doctor may recommend trying eye drops or artificial tears to moisten your eyes. […] The Mayo Clinic recommends trying over-the-counter saliva substitutes to treat dry mouth. […] Your doctor may recommend following the Mediterranean diet. This dietary plan incorporates plenty of fruits, veggies, whole grains, and healthy fats.

• #44 Primary biliary cholangitis – Wikipedia

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

  Pruritus is a common symptom in people with PBC. First-line treatment of pruritus consists of anion-exchange resins, such as cholestyramine, colestipol, or colesevalam. These anion-exchange resins are nonabsorbed, highly positively charged substances that bind bile acids, which are negatively charged anions. Anion-exchange resins relieve itching caused by excess bile acids in circulation by binding bile acids in the gut and facilitating elimination. Bloating or constipation may occur with anion-exchange resins. Cholestyramine may affect absorption of UDCA; if cholestyramine is necessary, it should be taken at least 60 minutes before or 4 hours after UDCA is taken. […] Treatment options for pruritus that does not improve with anion-exchange resins include: rifampicin, naltrexone, or sertraline. Rifampicin may rarely cause drug induced liver injury and should be avoided if serum bilirubin is elevated (greater than 2.5 mg/dL). Liver enzymes should be monitored after starting rifampin. Opioid antagonists may cause a self-limited opioid withdrawal like reaction, with abdominal pain, elevated blood pressure, tachycardia, goose bumps, nightmares, and depersonalization. To avoid such reactions, the dose should start low and gradually be increased. […] In patients with advanced liver disease, the only curative therapy is liver transplant. Outcomes are favourable, with five-year patient survival rates better than for most other indications for LT (80-85%).

• #45

  https://link.springer.com/article/10.1007/s12072-021-10276-6

  It is recommended that all PBC patients should be evaluated for osteoporosis, especially in postmenopausal women. […] No effective therapy for fatigue is available at this moment. […] The long-term post-transplant survival is relatively optimistic in PBC patients, with a 5-year survival rate of 80-85%. […] It is recommended that liver transplantation should be considered in patients with decompensated cirrhosis, MELD ≥ 15, Mayo Risk Score ≥ 7.8, or severe, intractable pruritus.

• #46 Primary Biliary Cirrhosis: New Options | Borland Groover

  https://borlandgroover.com/patient-resources/gastroenterology/gi-education/primary-biliary-cirrhosis-new-options

  Important supportive treatments are available for the complications of PBC. For fatigue, patients should be assessed for anemia, hypothyroidism, depression and sleep disorders. Modafinil at a dose of 100-200 mg/day has been associated with decreased daytime somnolence. In addition to UDCA, pruritus may be treated with bile acid binding agents such as cholestyramine at 4-16 grams daily. Rifampin at a dose of 150 mg daily to twice daily has been shown to relieve pruritus. Opioid receptor antagonists including naloxone infused IV and naltrexone orally have been shown by meta-analysis to significantly reduce pruritus. Naltrexone is often started at a low dose such as 12.5 mg daily with a slow titration over several weeks to 50 mg daily to reduce the chance of producing an opioid withdrawal reaction.

• #47 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Certain complications are commonly associated with primary biliary cholangitis. Your healthcare team may recommend: […] Vitamin and mineral supplements. […] Medicine to lower cholesterol. […] Medicines to treat bone loss. […] Treatment for increased pressure in the portal vein, called portal hypertension.

• #48 PBC: Treatment and Complications

  https://www.webmd.com/digestive-disorders/primary-biliary-cirrhosis-treatment

  Doctors are researching other drugs to see if they can help. Some, like colchicine and methotrexate, suppress your immune system. Other studies suggest that fenofibrate or prednisone might help. Your doctor can help you decide whats best for you. […] Treatments usually keep primary biliary cholangitis from getting worse. If they dont, your liver could start to fail. If that happens, your doctor will discuss a liver transplant. In this surgery, your liver is removed and replaced with a healthy, donated liver. […] Medication is the main way to treat primary biliary cholangitis. Your doctor will work with you to find the best drug treatment plan. But you can also make simple changes in your daily life that will help ease your symptoms. […] Your doctor can suggest a healthy meal plan. It’s smart to eat a balanced diet rich in fresh fruits, vegetables, and whole grains. […] When bile can’t move around the way it should, it can be hard for your body to absorb fat. You might not get enough of vitamins A, D, E, and K — the ones that dissolve in fat. Your doctor might suggest you take these vitamins as replacement therapy.

• #49 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Certain complications are commonly associated with primary biliary cholangitis. Your healthcare team may recommend: […] Vitamin and mineral supplements. […] Medicine to lower cholesterol. […] Medicines to treat bone loss. […] Treatment for increased pressure in the portal vein, called portal hypertension.

• #50 Primary biliary cholangitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/diagnosis-treatment/drc-20376880

  Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] Antihistamines are commonly used to reduce itching. […] Cholestyramine is a powder that may stop itching. […] Rifampin is an antibiotic that may stop itching. […] Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. […] Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce itching.

• #51 Treating advanced primary biliary cirrhosis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/primary-biliary-cholangitis/treating-advanced-primary-biliary-cholangitis/

  Ursodeoxycholic Acid worked really well for around ten years although there was cirrhosis there was no deterioration. […] Cirrhosis can cause a number of other serious health problems. For some patients the best way to deal with these may be to have a liver transplant. This means your damaged liver is removed during surgery and replaced with a healthy, donor liver. […] Your doctor may suggest it if your liver damage is so severe as to be life-threatening. But with PBC, some people have such severe itching that it really impacts their quality of life. In this situation, your doctor may also suggest a transplant. […] Liver transplant is usually very successful these days. Although its possible for PBC to come back in your transplanted liver, it tends to be very slow to develop and unlikely to become advanced.

• #52 Primary biliary cholangitis – Wikipedia

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

  Pruritus is a common symptom in people with PBC. First-line treatment of pruritus consists of anion-exchange resins, such as cholestyramine, colestipol, or colesevalam. These anion-exchange resins are nonabsorbed, highly positively charged substances that bind bile acids, which are negatively charged anions. Anion-exchange resins relieve itching caused by excess bile acids in circulation by binding bile acids in the gut and facilitating elimination. Bloating or constipation may occur with anion-exchange resins. Cholestyramine may affect absorption of UDCA; if cholestyramine is necessary, it should be taken at least 60 minutes before or 4 hours after UDCA is taken. […] Treatment options for pruritus that does not improve with anion-exchange resins include: rifampicin, naltrexone, or sertraline. Rifampicin may rarely cause drug induced liver injury and should be avoided if serum bilirubin is elevated (greater than 2.5 mg/dL). Liver enzymes should be monitored after starting rifampin. Opioid antagonists may cause a self-limited opioid withdrawal like reaction, with abdominal pain, elevated blood pressure, tachycardia, goose bumps, nightmares, and depersonalization. To avoid such reactions, the dose should start low and gradually be increased. […] In patients with advanced liver disease, the only curative therapy is liver transplant. Outcomes are favourable, with five-year patient survival rates better than for most other indications for LT (80-85%).

• #53 Primary Biliary Cholangitis (PBC) – Hepatic and Biliary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/fibrosis-and-cirrhosis/primary-biliary-cholangitis-pbc

  For patients who do not respond adequately to ursodeoxycholic acid and do not have access to obeticholic acid, bezafibrate may be used in combination with ursodeoxycholic acid. […] Pruritus may be controlled with cholestyramine 4 to 16 g/day. […] Patients with fat malabsorption due to bile salt deficiency should be treated with vitamin A, vitamin D, vitamin E, and vitamin K supplements. […] Liver transplantation has excellent results. […] AMA-negative PBC has similar treatment response to ursodeoxycholic acid.

• #54 Primary Biliary Cholangitis: What It Is, Symptoms, Treatment

  https://my.clevelandclinic.org/health/diseases/17715-primary-biliary-cholangitis-pbc

  Primary biliary cholangitis is a chronic and progressive condition that causes inflammation and, eventually, the destruction of the bile ducts that run through your liver. Without working bile ducts, bile backs up in your liver, causing liver damage. This can lead to cirrhosis of the liver. But medication can delay and sometimes prevent it. […] Theres no cure for PBC, but you can slow it down and improve your condition with medication. Ursodeoxycholic acid (UDCA) is a type of bile salt that can help clear bile from your liver and reduce liver damage. It works well for about half of people with PBC, especially in the early stages. For those who dont benefit from UDCA, doctors sometimes recommend a different bile salt called obeticholic acid. […] If medication doesnt improve your condition and your liver function continues to decline, your doctor may put you on the liver transplant waiting list. Liver transplant surgery has excellent results for people with PBC. Although, like other autoimmune diseases, PBC may return after your liver transplant, but it tends to progress much more slowly the second time around. Life expectancy after your transplant is normal.

• #55 Primary Biliary Cirrhosis: New Options | Borland Groover

  https://borlandgroover.com/patient-resources/gastroenterology/gi-education/primary-biliary-cirrhosis-new-options

  Liver transplantation is the treatment of last resort in primary biliary cirrhosis, however, when required, patients with PBC do better than most other etiologies of liver failure. PBC is currently the sixth leading cause of liver transplant in the U.S. (down from the most common cause in the mid 1980s). Approximately 20-25% of patients who undergo liver transplant for PBC have recurrent PBC in the graft after 10 years, however, recurrent disease does not affect graft survival. Referral for transplantation evaluation is considered for symptomatic patients with cirrhosis by biopsy, who have model for end stage liver disease (MELD) scores of 12-15 or who have hepatic decompensations such as encephalopathy, bleeding from varices, or the development of ascites.

• #56 Treating advanced primary biliary cirrhosis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/primary-biliary-cholangitis/treating-advanced-primary-biliary-cholangitis/

  Ursodeoxycholic Acid worked really well for around ten years although there was cirrhosis there was no deterioration. […] Cirrhosis can cause a number of other serious health problems. For some patients the best way to deal with these may be to have a liver transplant. This means your damaged liver is removed during surgery and replaced with a healthy, donor liver. […] Your doctor may suggest it if your liver damage is so severe as to be life-threatening. But with PBC, some people have such severe itching that it really impacts their quality of life. In this situation, your doctor may also suggest a transplant. […] Liver transplant is usually very successful these days. Although its possible for PBC to come back in your transplanted liver, it tends to be very slow to develop and unlikely to become advanced.

• #57 Treating advanced primary biliary cirrhosis – British Liver Trust

  https://britishlivertrust.org.uk/information-and-support/liver-conditions/primary-biliary-cholangitis/treating-advanced-primary-biliary-cholangitis/

  If you have decompensated cirrhosis, your doctor may suggest that you have regular tests to pick up any complications as early as possible, when they are easier to manage. […] Your doctor may treat this with tablets, reducing salt intake and advice about diet. If the fluid is making you very uncomfortable, you may have it drained into a bag through a needle and tubing. […] Treating these will in turn treat the encephalopathy. It is important to tell your doctor as encephalopathy can be life-threatening if not controlled. […] Your doctor or specialist nurse may suggest you see a dietician. You may benefit from diet supplementary drinks, such as Complan, Ensure or Fresubin.

• #58 Management of Primary Biliary Cholangitis: Current Treatment and Future Perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10081121/

  Up to 40% of patients with PBC have an incomplete response to UDCA, this patient population requires additional pharmacotherapies with the goal of inducing biochemical remission. […] Peroxisome proliferator-activated receptor agonists including bezafibrate, fenofibrate, and elafibranor have shown promise in inducing biochemical remission in PBC patients who are UDCA non-responders. […] Additional investigational therapies including fibroblast growth factor-19 agonists, farnesoid X receptor agonists, and NADPH oxidase 3 inhibitors are being explored as potential disease-modifying therapies. […] Therefore, additional therapeutics for the management of PBC, particularly for those with advanced fibrosis and UDCA non-responders, are urgently needed. […] Obeticholic acid has been shown to be effective in UDCA non-responders and currently is considered second-line therapy. However, it is contraindicated in cirrhotic patients who have evidence of portal hypertension. […] There is a great need for additional pharmacotherapy to induce disease remission in PBC patients who do not respond to UDCA. […] Fibroblast growth factor-19 agonists and NOX inhibitors are also under investigation for further DMT options.

• #59 Primary Biliary Cholangitis: New treatment goals and novel salvage therapy | World Gastroenterology Organisation

  https://www.worldgastroenterology.org/publications/e-wgn/e-wgn-expert-point-of-view-articles-collection/primary-biliary-cholangitis-new-treatment-goals-and-novel-salvage-therapy

  The first line of treatment for PBC is a trial of weight-based (13 mg/kg/day in divided doses) UDCA therapy. […] If failure to reach treatment goals still persists after addition of either of these agents, either OLT or clinical trials are considered. […] Recent studies point towards normal ALP levels as the desired biochemical goal for effective PBC therapy. […] These findings carry even more weight when we look at the high number of promising therapeutic options, with many different mechanisms of action, currently being investigated. […] Although data is still limited in many of the novel PBC treatments mentioned above, early clinical trials have shown remarkable potential.

• #60 Can a Nanoparticle Drug Cure Primary Biliary Cholangitis?

  https://www.uvaphysicianresource.com/can-nanoparticle-cure-primary-biliary-cholangitis/

  For patients with primary biliary cholangitis (PBC), treatments can often keep the disease under control, preventing progression to cirrhosis and end-stage liver disease. […] The current state of PBC treatment is not ideal. While long-term use of ursodiol (ursodeoxycholic acid) can delay or prevent liver damage in people with PBC, it doesn’t work for everyone. […] Another drug, obeticholic acid, may also be used, but it has a fair number of side effects. […] Novel peroxisome proliferator-activated receptors (PPAR) agonists also are being developed for treating PBC and appear to put the disease into remission for some patients, but are not yet widely available, he adds. […] In a recently launched phase 2a, first-in-human trial, researchers are studying CNP-104. This nanoparticle-based drug is designed to desensitize the immune system.

• #61 Can a Nanoparticle Drug Cure Primary Biliary Cholangitis?

  https://www.uvaphysicianresource.com/can-nanoparticle-cure-primary-biliary-cholangitis/

  The nanoparticles used in the trial were developed by COUR Pharmaceutical. The double-blind, placebo-controlled study is enrolling 40 patients at centers around the U.S., including UVA Health. The drug is being studied in PBC patients who have had an incomplete response to ursodiol. […] If the drug is safe and effective, investigators expect that no further treatment will be needed.

• #62 New Primary biliary cholangitis (PBC) treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=32

  Management of Primary Biliary Cholangitis requires a personalized approach, taking into account disease severity, patient response to standard therapies, and potential side effects. While UDCA remains the cornerstone of PBC treatment, second-line therapies, off-label medications, experimental treatments, and liver transplantation provide additional options for patients who do not adequately respond to standard care.

• #63 Overview of the management of primary biliary cholangitis – UpToDate

  https://www.uptodate.com/contents/overview-of-the-management-of-primary-biliary-cholangitis

  Overview of the management of primary biliary cholangitis […] Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by an ongoing immunologic attack on the intralobular bile ducts that leads to chronic cholestasis and cirrhosis. The terminology was changed from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disorder and its natural history. The prognosis of patients with PBC has improved greatly because of its diagnosis at earlier stages and the widespread use of ursodeoxycholic acid as treatment. […] The goals of management are: […] • Suppression of the underlying pathogenic process (ie, destruction of small intralobular hepatic bile ducts) […] • Treatment of symptoms that result from chronic cholestasis […] • Management of complications that result from chronic cholestasis […] […] […] Approach to slowing disease progression for patients with primary biliary cholangitis

• #64 Holistic Patient Care in Primary Biliary Cholangitis: Managing Both the Disease and the Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/holistic-patient-care-in-primary-biliary-cholangitis-managing-both-the-disease-and-the-symptoms-j070122/

  In the progressive, immune-mediated liver disease primary biliary cholangitis (PBC), the intrahepatic bile ducts are gradually destroyed over several years. The primary biochemical means to diagnose PBC, and assess progression and treatment response, is serum alkaline phosphatase (ALP). First-line therapy for PBC is ursodeoxycholic acid (UDCA), which has been shown to improve biochemical indices of PBC and slow disease progression. […] The only fully approved first-line medication for PBC is UDCA, using a therapeutic dose of 1315 mg/kg/day, which should be initiated for all patients with PBC. For patients with an inadequate response to, or who are intolerant of, UDCA, obeticholic acid is approved as an adjuvant second-line therapy or monotherapy. […] UDCA has been shown to improve biochemistry and long-term outcomes with a reduced need for liver transplantation and death. […] The primary therapy for PBC is UDCA, the biochemical response to which can be monitored using liver blood tests such as ALP. Such tests can be used to assess response to treatment and risk stratify patients, improving identification of patients who may benefit from second-line therapies.

• #65 Update on Emerging Treatment Options for Primary Biliary Cholangitis | HMER

  https://www.dovepress.com/update-on-emerging-treatment-options-for-primary-biliary-cholangitis-peer-reviewed-fulltext-article-HMER

  Combination therapy utilizing UDCA, budesonide and mycophenolate mofetil (MMF) have been evaluated in a small study involving 15 patients with severe PBC with interface hepatitis and not meeting diagnostic criteria for autoimmune hepatitis (AIH). […] Clinical symptoms of PBC include fatigue and pruritus, both of which can be debilitating and lead to decreased quality of life. Both FDA approved medications for PBC, UDCA and OCA, have no impact on management of these symptoms. Therefore, each symptom must be addressed independently. Unfortunately, treatment options are limited and liver transplantation may be the only cure for many. […] Although several treatment options have been investigated, UDCA remains first-line therapy and has proven efficacious in normalizing serum biochemistries and halting the progression of fibrosis leading to longer transplant-free survival. Though OCA was FDA approved for combination therapy in patients with inadequate response to UDCA, the significant side effect of pruritus may limit its use in patients already afflicted with severe symptoms. The pipeline remains promising as multiple trials are underway to address patients with inadequate or non-response to therapy with UDCA.

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