Materiały źródłowe

• #1 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. […] 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. A baseline bone density is currently recommended at the time of initial diagnosis.

• #2 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

  Primary biliary cholangitis (PBC; formerly known as primary biliary cirrhosis) is an autoimmune liver disorder characterized by the progressive destruction of intrahepatic bile ducts, leading to cholestasis, cirrhosis, and liver failure. […] Treatment includes ursodeoxycholic acid, obeticholic acid, bezafibrate, cholestyramine (for pruritus), supplementary fat-soluble vitamins, and, ultimately for advanced disease, liver transplantation. […] All alcohol use and hepatotoxic medications should be stopped. Ursodeoxycholic acid decreases liver damage, prolongs survival, and delays the need for liver transplantation. […] Stop use of hepatotoxins (including alcohol), and treat with ursodeoxycholic acid, which may delay the need for transplantation. Obeticholic acid is the second-line treatment. Fibrate can be added to ursodeoxycholic acid. Obeticholic acid and fibrate should be avoided in decompensated cirrhosis. […] Transplantation is indicated for decompensated liver disease (uncontrolled variceal bleeding, refractory ascites, intractable pruritus, hepatic encephalopathy).

• #3 Primary Biliary Cholangitis (PBC) — Irish Liver Foundation

  https://www.liverfoundation.ie/primary-biliary-cholangitis-pbc

  Currently, there is no known way to prevent PBC since its exact cause is not understood. […] However, early diagnosis and continued treatment can help manage the disease and minimize its impact on liver function.

• #4 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. […] But medication can delay and sometimes prevent it. […] Fortunately, medication helps slow the progress of the disease, and not everyone will reach this stage. […] Theres no cure for PBC, but you can slow it down and improve your condition with medication. […] With earlier diagnosis and treatment, you can often prevent or at least delay the later stages and complications of the disease. […] Many people are able to effectively control their symptoms with medications, although fatigue in PBC continues to be difficult to treat.

• #5

  https://www.nhs.uk/conditions/primary-biliary-cholangitis-pbc/treatment/

  The aim of treatment for PBC is to slow down the liver damage and reduce your symptoms. […] Following this advice could: help prevent PBC getting worse. […] A liver transplant may be recommended if it’s thought the liver damage may eventually put your life at risk. […] Planning for a liver transplant often begins before significant damage to the liver has taken place. […] Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue. […] Medicines that suppress the immune system are very effective at preventing this, but they need to be taken for life to reduce this risk.

• #6 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

  Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease that may progress to fibrosis or cirrhosis. Treatment options are currently limited. Ursodeoxycholic acid (UDCA) remains first-line therapy and has been proven to normalize serum biochemistries, halt histologic disease progression, and lead to patient survival comparable to the general population. […] UDCA has also been studied for use in recurrent PBR (rPBC) after LT. […] UDCA was dosed at 10-15 mg/kg/day. They found that rPBC was significantly lower in the UDCA group compared to patients who were not started on prophylaxis (21% vs 62%, P=0.004). However, they also noted no significant improvement in long-term patient or graft survival. […] The standard of care for PBC includes treatment with 13-15 mg/kg/day of UDCA in divided doses.

• #7 Primary Biliary Cholangitis – Liver Foundation

  https://liver.org.au/your-liver/liver-diseases/primary-biliary-cholangitis-2/

  PBC is a rare medical condition. PBC stands for primary biliary cholangitis. It’s a medical condition that affects the small bile ducts in the liver. […] The mainstay of treatment is a medication called ursodeoxycholic acid which is a derivative of a bile salt that we make in our own bodies that is a healthy bile salt. It is a well-tolerated medication, and most people will be prescribed this long term. […] There’s no cure for PBC, but medications can be used to slow it down and prevent complications. Medicines you might be given include: Ursodeoxycholic acid or UDCA capsules. These help the liver work properly by moving bile out of the liver and into the small intestine. This medication can prevent the PBC developing into liver failure. You will need to take it every day for the rest of your life.

• #7 Primary Biliary Cholangitis – Liver Foundation

  https://liver.org.au/your-liver/liver-diseases/primary-biliary-cholangitis-2/

  If you start to develop liver cirrhosis, this will also need to be treated. […] Eventually, the PBC can lead to liver failure. Having a liver transplant is usually very successful. Sometimes the PBC can come back, but it rarely leads to liver cirrhosis after a transplant. […] I would encourage people who are diagnosed with PBC to think in a very positive manner. There are good medications, there’s increasing availability of newer medications, and we are very good at treating the complications of a condition today. […] If you’ve been diagnosed with PBC, there are a few things you can do to make yourself feel better. Cut down on salt. This will help with fluid retention and swelling. Cut down on the fat in your diet, especially if you are overweight. Drink plenty of water. Cut out alcohol. Don’t take any drugs that can harm the liver (including illegal drugs, alternative medicines and liver tonics). Do more exercise. Walking is great. Try to do some weight training as well to protect your bones. De-stress if you can. Stop smoking. Look after your skin, especially if you are very itchy. Get your teeth checked regularly, especially if you have a dry mouth.

• #8 New treatment option for Primary Biliary Cholangitis (PBC) | This Changed My Practice (TCMP) by UBC CPD

  https://thischangedmypractice.com/new-treatment-primary-biliary-cholangitis/

  UDCA remains first-line therapy for PBC with a long record of use and generic preparations but in non-responders, OCA treatment should be considered. […] Patients treated with UDCA should have biochemical monitoring every 3-6 months. […] If these criteria are not met, specifically if ALP remains elevated over 200 IU/L despite UDCA treatment, it is prudent to first consider patient adherence, concomitant liver disease, and co-administration with bile acid sequestrants, which can decrease absorption of UDCA. If there is no other cause identified, a diagnosis of inadequate response to UDCA can be made. […] OCA should be used in combination with UDCA in patients with inadequate UDCA response. […] OCA dosing is based on the existing degree of liver impairment. […] Treatment in patients with moderate to severe liver impairment is not well established as there is a risk of dose-dependent hepatotoxicity in these populations; treatment should be initiated and monitored by a hepatologist. […] Treatment with OCA can worsen pruritus. […] OCA is more costly than UDCA but has Limited Coverage through PharmaCare in British Columbia.

• #9 Primary Biliary Cirrhosis (PBC) | Autoimmune Liver Diseases | UPMC

  https://www.upmc.com/services/digestive-disorders-center/services/liver-diseases/conditions/autoimmune-liver-diseases/primary-biliary-cholangitis

  Doctors can’t cure PBC. But they can treat you to help stop or slow further liver damage. […] The goal of treating PBC is to prevent liver damage. You’ll see your doctor for routine physical exams and blood tests to check your liver’s function. […] Healthy habits are especially vital when you have PBC. […] Your doctor will suggest that you: Eat high-calcium foods. Exercise. Avoid alcohol. Limit salt. […] Your doctor might prescribe certain medications to help slow liver damage from PBC. […] These drugs include: Ursodiol an acid that helps break down cholesterol in the liver. Obeticholic acid which may help decrease inflammation in the liver. […] Some people with PBC don’t have enough vitamins A, D, E, and K in their bodies because the liver can’t absorb them. You might take supplements to help replace these vitamins. […] Over time, PBC may damage the liver so much that you experience liver failure or end-stage liver disease. […] Transplant surgery, in which doctors replace a diseased liver with a donated healthy one, is the only treatment for liver failure.

• #10 Primary Biliary Cholangitis – PBC | Choose the Right Test

  https://arupconsult.com/content/primary-biliary-cirrhosis

  Various criteria may be used to assess the biochemical response to ursodeoxycholic acid (UDCA) therapy. Noninvasive tests, including bilirubin, ALP, AST, albumin, and platelet count tests, can be used at baseline and for ongoing monitoring. Transient elastography may also be used to assess response to treatment. In patients with cirrhosis complications, persistent markers of severe disease, and/or severe medically resistant pruritus, consider evaluation for liver transplant. […] Evaluation of biochemical response to UDCA is recommended 12 months after treatment initiation to determine whether second-line therapy (obeticholic acid) should be considered. […] Guidelines also recommend periodic monitoring of liver biochemistry and monitoring for related complications, per the table below. […] Lifelong follow-up is recommended.

• #10 Primary Biliary Cholangitis – PBC | Choose the Right Test

  https://arupconsult.com/content/primary-biliary-cirrhosis

  Primary biliary cholangitis (PBC), previously referred to as primary biliary cirrhosis, is an autoimmune liver disorder characterized by chronic, progressive cholestatic disease. If untreated, PBC leads to cirrhosis, or scarring of the entire liver, which can result in liver failure. […] Although earlier detection and intervention have reduced the need for liver transplantation in patients with primary biliary cholangitis (PBC), the British Society of Gastroenterology (BSG) recommends consideration of liver transplantation in all patients with bilirubin 50 mol/L or with pruritus that is refractory to all medical therapy. […] In patients with advanced primary biliary cholangitis (PBC), fat and fat-soluble vitamins (A, D, E, and K) may not be well absorbed. Laboratory testing for vitamin deficiency and supplementation should be considered.

• #11 Primary biliary cholangitis, a rising health burden

  https://www.explorationpub.com/Journals/ei/Article/1003165

  Patients with PBC should receive age-specific recommended vaccinations. HAV and HBV vaccines should be administered if the patient does not have immunity to them. […] Patients should be counseled about smoking cessation, alcohol abstinence, illicit drug use, adequate calcium and vitamin D supplementation, exercise, fall prevention, and HCC surveillance. Bone mineral density testing is recommended every 2-3 years. […] Prophylactic use of UDCA after L.T. was associated with reduced risk of recurrence in a recent meta-analysis of 15 retrospective cohort studies including up to 1,727 liver transplanted patients, cyclosporine-containing immunosuppressive regimens are associated with a lower risk of recurrence.

• #12 Primary Biliary Cholangitis (PBC)

  https://rare-liver.eu/patients/understand-your-diagnosis/primary-biliary-cholangitis-pbc/

  How PBC is monitored is dependent on the severity of the disease. […] Patients suffering from PBC can have an increased risk of osteoporosis (reduced bone density). Therefore, bone density tests (osteodensitometry) and preventive measures may be needed. […] There is no special diet for PBC. The recommendation will be to maintain a healthy, well-balanced diet, as for the general population. […] Vaccinations against hepatitis A and B viruses and pneumococcal infections (bacteria) are recommended, as well for COVID-19.

• #13 Primary Biliary Cholangitis (PBC) Information & Treatment

  https://www.columbiadoctors.org/health-library/condition/primary-biliary-cholangitis-pbc/

  Treatment for PBC focuses on: […] Preventing and treating the complications of the disease. […] Preventing other conditions that may cause more liver damage. […] Because people with PBC have a high risk of getting osteoporosis, your doctor may suggest that you take calcium and vitamin D supplements. […] Your doctor may also have you take a medicine called a bisphosphonate.

• #14

  https://myhealth.alberta.ca/Health/pages/conditions.aspx?hwid=aa89995spec

  Treatment for PBC focuses on: […] Preventing and treating the complications of the disease. […] Preventing other conditions that may cause more liver damage. […] Because people with PBC have a high risk of getting osteoporosis, your doctor may suggest that you take calcium and vitamin D supplements. […] Your doctor may also have you take a medicine called a bisphosphonate. […] There are things you can do at home. For example, if your doctor prescribed medicines, take them as instructed. […] Don’t drink alcohol. […] And stay up to date on your vaccines.

• #15 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

  Pruritus is another common symptom of PBC, reported by up to 80% of patients. There are currently multiple medication options for PBC associated pruritus. […] First-line treatment of PBC associated pruritus is bile acid resins. […] Second-line therapy for PBC pruritus includes rifampicin and opioid antagonists. […] Naltrexone, an oral opioid antagonist has been shown through two small randomized controlled trials to be an effective option for the management of pruritus in patients with cholestatic liver disease.

• #16 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: […] prevent complications or delay them for as long as possible. […] If your PBC can’t be controlled with medicine, then a liver transplant is also an option. […] If you have itching, your doctor will suggest a medicine called colestyramine. This helps to get rid of the bile acids that are causing the itching. […] If you can’t take colestyramine or it isn’t helping, your doctor may suggest other medicines for itching. […] Unfortunately, there are no medicines your doctor can suggest that can help with fatigue. But they can check for other conditions that may be making fatigue worse.

• #17 Primary Biliary Cholangitis: Symptoms & Causes

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

  As the ability of the liver to function decreases over time, complications associated with cirrhosis will need to be addressed and treated. Screening for varices and liver cancer is often recommended. […] Maintaining a healthy lifestyle can help people with Primary Biliary Cholangitis (PBC) feel better, as well as relieve or prevent some symptoms associated with the disease. Upon diagnosis, your doctor may suggest the following: Start a reduced sodium diet if you have fluid overload (edema or ascites), Reduce fat intake if you are overweight or have fatty liver, Drink plenty of water, Avoid or lower intake of alcohol, Avoid undue stress when possible, Start exercising, particularly walking, Stop smoking, Maintain good skin care, Get regular dental examinations. […] While there is no cure, people are having good results slowing disease progression and living longer without complications by adhering to their medication regimen and maintaining a healthy lifestyle.

• #18 Primary Biliary Cholangitis (PBC): Symptoms & Treatment

  https://www.health.com/primary-biliary-cholangitis-8648831

  Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is a chronic and progressive disease that damages and can eventually destroy the bile ducts of the liver. […] While there may be no way to completely prevent primary biliary cholangitis, certain lifestyle and dietary choices may help manage the condition. Consider these methods: Eat a well-balanced diet high in nutrients from fresh vegetables, fruit, and lean protein sources; Take vitamins A, D, E, and K to prevent bone loss due to liver damage; Stop or severely limit alcohol use; Avoid undercooked shellfish, fish, meat, and unpasteurized milk; Avoid smoking; Avoid certain medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs) like Advil (ibuprofen).

• #19 Primary biliary cholangitis (PBC) – Swiss HePa

  https://www.swisshepa.org/en/liver-diseases/primary-biliary-cholangitis-pbc/

  Primary biliary cholangitis (formerly primary biliary cirrhosis) one of the rarest autoimmune liver diseases. This rare liver disease affects more women than men. With targeted treatments, up to two-thirds of those affected today do not go on to develop cirrhosis. […] As with every chronic liver disease, a diagnosis of PBC means avoiding alcohol and nicotine is recommended.

• #20 Primary Biliary Cholangitis (PBC): Symptoms and Treatment

  https://patient.info/digestive-health/abnormal-liver-function-tests-leaflet/primary-biliary-cholangitis

  Primary biliary cholangitis (PBC) is a condition that slowly damages the bile ducts in the liver, leading to liver damage and, sometimes, liver scarring (cirrhosis). […] If you have primary biliary cholangitis (PBC), you may find that you are no longer able to cope with drinking alcohol. […] It is advisable to take particular care around alcohol consumption if you know that you have a developing liver disease like PBC. […] You should always remember to tell a doctor or a pharmacist that you have PBC before you start taking any medication. […] Worsening liver disease affects your ability to absorb a balanced diet, and chronic tiredness will reduce your fitness. […] It is sensible to seek the advice of a dietician to help to ensure a balanced diet, and to assess whether you need food and vitamin supplements, particularly the fat soluble vitamins A, D, E and K. […] Physical activity will help you stay fitter for longer. Being physically active for at least 30 minutes every day of the week is a healthy target.

• #21 Primary Biliary Cholangitis: Symptoms, Causes, Diagnosis, and Treatment

  https://www.everydayhealth.com/liver-disease/primary-biliary-cholangitis/

  The cause of primary biliary cholangitis isnt completely understood. Not smoking and limiting exposure to toxic chemicals that may be present in certain work environments may reduce the risk of developing the disease. […] While lifestyle changes wont treat the disease directly, they can help to slow its progression, limit or prevent complications, and improve quality of life. […] People with PBC should stop drinking alcohol (especially heavy alcohol use). […] Regular exercise is recommended. […] See a liver specialist regularly to manage your medications and address any health issues you may have.

• #22 Primary Biliary Cirrhosis Life Expectancy, Symptoms, Treatment

  https://www.medicinenet.com/primary_biliary_cirrhosis_pbc/article.htm

  Can primary biliary cirrhosis be prevented? […] Since the cause of the disease is not known, it is not possible to prevent PBC. However, medications may be able to slow the progression of the disease and control the symptoms. […] Should a person develop PBC, all attempts should be made to prevent further potential damage. This may include limiting alcohol intake and avoiding the use of medications that contain acetaminophen (Tylenol, Panadol), and cholesterol-lowering agents.

• #23 Primary Biliary Cholangitis (PBC) – HealthyWomen

  https://www.healthywomen.org/condition/primary-biliary-cholangitis-pbc

  The exact cause of primary biliary cholangitis (PBC) is unknown. However, it is known that PBC is not caused by alcohol or illegal-drug use. It is an autoimmune disease that occurs in genetically susceptible individuals. The body’s immune system mistakenly attacks and destroys its own cells in this case, the cells of the intrahepatic bile ducts. Some people diagnosed with PBC may also have one or more other autoimmune diseases. […] PBC is an autoimmune disease that cannot be prevented or cured, but there is treatment available to slow disease progression. […] Maintaining a healthy lifestyle can help people with PBC feel better, as well as relieve or prevent some symptoms associated with the disease. Once you are diagnosed, your health care provider may suggest the following: Start a reduced-sodium and/or low-fat diet. Avoid or lower alcohol consumption. Lower caffeine intake. Avoid stress when possible. Start exercising, particularly walking. Stop smoking. Maintain good skin care. Get regular dental examinations.

• #24 Sclerosing cholangitis vs. Primary biliary cholangitis

  https://www.medicalnewstoday.com/articles/sclerosing-cholangitis-vs-primary-biliary-cirrhosis

  Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is an autoimmune disease that damages the small bile ducts inside the liver. […] Over time, this damage leads to bile buildup, liver scarring, and possibly liver failure. […] The main treatment is ursodeoxycholic acid (UDCA), which helps slow the disease and improve liver function.

• #25 Shifting Treatment Goals, Timelines in Primary Biliary Cholangitis

  https://www.hcplive.com/view/shifting-treatment-goals-timelines-in-primary-biliary-cholangitis

  In recognition of the US Food and Drug Administration accelerated approval of Gilead’s seladelpar (Livdelzi) and other recent evolutions in primary biliary cholangitis (PBC), the latest HCPLive Special Report spotlights a conversation between a trio of subject matter experts on the latest advancements in PBC and their implications for disease management. […] Ursodeoxycholic acid (UDCA) has long been the mainstay of treatment for PBC and continues to be the only first-line therapy. However, many patients do not respond to or are unable to tolerate UDCA, underscoring the need for more treatment options to prevent progression to cirrhosis and eventual liver failure as well as to improve symptoms most negatively impacting patients. […] Now, current approaches aim not only to prevent disease progression but also to normalize liver function tests and improve overall patient well-being. […] This shift underscores the necessity of introducing second-line agents sooner if patients do not show significant improvement.

• #26 Primary Biliary Cholangitis (PBC) | NewYork-Presbyterian

  https://www.nyp.org/digestive/liver-diseases/primary-biliary-cirrhosis-pbc

  Primary biliary cholangitis (PBC) is a rare, chronic, progressive inflammatory disease that slowly destroys the small bile ducts in the liver and may eventually cause the liver to stop functioning properly. […] While there is no cure for primary biliary cholangitis, NewYork-Presbyterian provides treatments designed to slow the progression of your disease, relieve your symptoms, and prevent or treat complications so you can live a longer, more comfortable life. […] Doctors may prescribe the drug ursodeoxycholic acid (UDCA), a naturally occurring bile acid, to increase the flow of bile and reduce inflammation of the bile ducts. […] If your PBC becomes so advanced that it cannot be effectively managed with other treatments, you may need a liver transplant. […] Your doctors will monitor your bone density because people with PBC are also at risk for osteoporosis.

• #27 Primary biliary cholangitis – USZ

  https://www.usz.ch/en/disease/primary-biliary-cholangitis/

  Primary biliary cholangitis: prevention, early detection, prognosis […] PBC progresses very differently. The course of PBC can be favorably influenced with medication. Some patients with a particularly mild course have only a few changes in their liver 20 years after the initial diagnosis. In others, the disease progresses much faster. Special blood tests can determine factors that indicate a better or worse prognosis. If there is a risk of very rapid and/or very severe damage to the liver, a liver transplant is the treatment of choice. Around two thirds of all PBC patients do not develop cirrhosis. They can grow old with their disease. […] PBC cannot be treated causally. It remains a lifelong companion. However, the course and severity of the disease can be favorably influenced by a whole range of medications. In some cases, PBC even comes to a complete standstill. Fewer and fewer people with PBC need a transplant today. […] If you are ill, you should avoid any further stress that impairs the function of the liver: Avoid alcohol, Avoid medications that are broken down by the liver (painkillers, sleeping pills) and Switch to a low-fat diet to reduce unpleasant diarrhea.

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