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. […] There is no cure for Primary Biliary Cholangitis (PBC), however, there are medications that can help slow disease progression and manage symptoms. […] Liver transplantation is considered when medical treatment no longer sufficiently controls the disease. […] 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.

• #2 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. […] Management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. […] Disease management focuses on initiation of UDCA for all patients and risk stratification based on baseline and on-treatment factors, including in particular the response to treatment. […] We recommend oral UDCA at 1315mg/kg/day is used as the first-line pharmacotherapy in all patients with PBC. If tolerated, treatment should usually be life-long. […] We recommend all patients should be evaluated for the presence of symptoms, particularly fatigue and itch. Clinicians should recognise that severity of symptoms does not correlate with stage of disease.

• #3

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  PBC is a chronic disease that frequently leads to cirrhosis, liver failure and is a common indication for liver transplantation. […] In recent years, there have been important advances in PBC. For example, the designation primary biliary cirrhosis was deemed no longer accurate since, with early diagnosis and treatment, many patients do not progress to cirrhosis. Therefore, to adequately describe the histologic hallmark of dense inflammatory infiltrates around damaged intralobular bile ductules, cholangitis has replaced cirrhosis, while retaining the acronym PBC. […] Because of important changes in the PBC landscape, and a number of unanswered questions, the American College of Gastroenterology (ACG) Institute for Clinical Research Education and the Chronic Liver Disease Foundation (CLDF) have collaborated to provide a practical guidance document for practicing gastroenterologists to help with their clinical practice. […] In summary, this guidance document for PBC, intended for practicing healthcare providers, was developed by a panel of hepatology experts and supported by the ACG Institute to provide specific recommendations about clinically relevant topics in PBC based on the most recent evidence as well their extensive experience and clinical expertise.

• #4 Primary biliary cirrhosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/primary-biliary-cirrhosis

  Primary biliary cholangitis (previously known as primary biliary cirrhosis) is characterised by the chronic inflammation and scarring of the bile ducts within the liver. […] There is no cure, but treatment can help slow the progression of disease and ease associated symptoms. […] The disease is usually diagnosed later in life, between the ages of 35 and 60 years. There is no cure, but treatment can slow the progression of the disease and alleviate symptoms. […] Fortunately, most people with PBC don’t ever experience this degree of cirrhosis. […] As a result, this disorder is now called primary biliary cholangitis, which refers to inflammation of the bile ducts. The older term primary biliary cirrhosis is no longer recommended. […] There is no cure for PBC, but treatment can help slow the progression of disease and associated symptoms. Options include: medication, particularly ursodeoxycholic acid (Ursofalk) for patients who do not respond, obeticholic acid (not available yet in Australia) and fibrates can be considered. […] Regular weight bearing exercise (such as walking or weight training) and calcium or vitamin D supplementation to reduce the risk of osteoporosis. […] Liver transplant, in severe cases.

• #5 Primary Biliary Cholangitis: Symptoms & Causes

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

  Since people with Primary Biliary Cholangitis (PBC) are at a higher risk for osteoporosis, calcium and vitamin D are usually prescribed. […] 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). […] Keep in mind that Primary Biliary Cholangitis (PBC) usually advances slowly over a period of years. […] Women are nine times more likely than men to develop Primary Biliary Cholangitis (PBC), meaning that women make up about 90% of Primary Biliary Cholangitis (PBC) cases. […] The disease most often develops during middle age and is usually diagnosed in people between the ages of 35 to 60 years.

• #6 Primary Biliary Cholangitis (Primary Biliary Cirrhosis): Practice Essentials, Background, Pathophysiology

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

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic disease of the liver, presumably autoimmune in nature, that leads to progressive cholestasis through intrahepatic bile duct destruction and often end-stage liver disease. The name change reflects the fact that cirrhosis occurs only in the late stage and therefore does not correctly identify patients with early-stage disease. […] PBC is most frequently a disease of women and occurs between the fourth and sixth decades of life. […] Pharmacologic treatment of PBC is as follows: Ursodeoxycholic acid (UDCA) is the first line medication used to slow the progression of the disease. […] In patients with cirrhosis, an elevated bilirubin level is an ominous sign of disease progression, and liver transplantation must be considered. Liver transplantation appears to be the only life-saving therapeutic option. […] Various therapeutic approaches have been implemented with variable results; in selected candidates, liver transplantation is the only treatment option for the terminal stages of the disease.

• #7 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. […] Primary biliary cholangitis (PBC) is a liver disease that affects the bile ducts that run through your liver. It slowly degrades those bile ducts, making it harder for bile to flow through. Bile backs up inside your liver, which damages the tissues. Scar tissue gradually replaces healthy tissue and your liver gradually loses its functionality. This is known as cirrhosis. PBC was formerly known as primary biliary cirrhosis. […] Primary biliary cholangitis is a chronic and progressive condition, which means it doesn’t go away and can get worse over time. It progresses slowly through several stages. At the beginning, you might not notice it at all. But in the end, it can cause liver failure, which is fatal without a liver transplant. Fortunately, medication helps slow the progress of the disease, and not everyone will reach this stage.

• #8

  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. […] Ursodeoxycholic acid (UDCA) is the main treatment for PBC. […] Once you start taking UDCA, it’s likely you’ll need to take it for the rest of your life. […] People who may need OCA will first need to be assessed by a specialist team with experience in treating PBC. […] Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC. […] No medicines are currently available to specifically treat fatigue associated with PBC. […] A liver transplant may be recommended if it’s thought the liver damage may eventually put your life at risk. […] Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue. […] But having a liver transplant does not always completely cure PBC.

• #9 Treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis) – NIDDK

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

  Doctors treat primary biliary cholangitis with medicines. […] Your doctor may prescribe ursodiol. Although ursodiol does not cure primary biliary cholangitis, it can slow the progression of liver damage. […] If you do not respond to ursodiol, your doctor may prescribe obeticholic acid. […] Your doctor may recommend over-the-counter medicines or prescribe medicines to treat symptoms of primary biliary cholangitis, such as itchy skin. […] Doctors may treat common complications of primary biliary cholangitis with medicines, dietary supplements, or changes in your diet and lifestyle. […] If primary biliary cholangitis leads to cirrhosis or portal hypertension, doctors can treat the health problems related to these conditions with medicines, minor medical procedures, and surgery. […] Your doctor will consider a liver transplant when your primary biliary cholangitis leads to liver failure. […] If you have primary biliary cholangitis, you can take steps to help prevent further liver damage.

• #10

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

  Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis. It’s a type of liver disease caused by damage to the bile ducts in the liver. PBC permanently damages the liver as tissue is replaced with scar tissue. As more scar tissue develops, the structure and function of the liver are affected. […] Treatment for PBC focuses on: […] Reducing symptoms. […] Preventing and treating the complications of the disease. […] Preventing other conditions that may cause more liver damage. […] Two medicines can be used to treat PBC. They are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA). Both medicines help move bile out of the liver into the small intestine. And OCA decreases the amount of bile acids made by the liver. […] 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.

• #11 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459209/

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. Prolonged hepatic cholestasis subsequently causes cirrhosis and portal hypertension. This activity reviews the evaluation and treatment of PBC and the importance of the interprofessional team in recognizing and managing patients with this condition. […] The management of primary biliary cholangitis is best done through an interprofessional team approach. The disorder has no cure and carries high morbidity and mortality. Treatment aims to slow disease progression and improve the quality of life. Besides the physicians, the nurse, mental health counselor, pharmacist, and physical therapist are critical in managing these patients. The nurse should educate the patient on the management of itching and the use of moisturizers. The dietitian should educate the patient on a healthy diet to prevent osteoporosis and deficiency of fat-soluble vitamins. The pharmacist should educate the patient on the Sicca syndrome and avoid dry eyes and mouth. Because of extreme fatigue, the patient should enroll in a physical therapy program or physical activity. Finally, the patient should see a mental health counselor as the disorder can result in premature death. The patient should be urged to join a support group and closely follow up with the healthcare provider.

• #12 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). […] Major quality of life (QoL)-impacting symptoms of PBC, including pruritus and fatigue, are demonstrated to be independent of disease severity. There is evidence confirming that these symptoms negatively impact a number of aspects, including emotional status, ability to work, and social life, for some patients. […] 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.

• #13 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/

  UDCA has been shown to improve biochemistry and long-term outcomes with a reduced need for liver transplantation and death. […] Biochemical liver tests can help healthcare professionals identify the best therapy option for patients with PBC. However, biochemistry will not necessarily be tackling symptom burden. […] In patients with PBC presenting moderatesevere symptoms, impacts include pruritus, fatigue, cognitive problems, social impairment, and emotional dysfunction. […] Cholestatic pruritus is one of the major symptoms of PBC, with around half of all patients experiencing this to some degree at any given time. […] Addressing pruritus symptoms is essential, highlighted Jones, because if you can roll the itch back, you can start to roll back several other difficult symptoms. […] Fatigue as a symptom of PBC can also greatly impact a patients QoL, with one study showing this occurred in 68.0% of patients.

• #14 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  For patients with the chronic progressive liver disease primary biliary cholangitis (PBC), personalised treatment is key to both preserving liver function and retaining health-related quality of life (HRQoL). […] According to symposium chair Gideon Hirshfield, such symptoms necessitate a patient-centric approach to PBC symptom management. […] Both symptoms need to be discussed and assessed at every visit to a healthcare professional (HCP), with the need for treatment guided by the patient. […] Christie concluded by discussing how we need to inform, educate, and empower patients to be involved in their own care journey. […] Hirschfield stressed how this patient-centric approach, where symptoms are readily addressed and managed in addition to starting disease-modifying therapy, could make a difference to HRQoL.

• #15

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  In patients with PBC and fatigue, it is important to exclude or identify and manage potential causes of fatigue such as anemia, depression, sleep disorders, hypothyroidism, and medications that can cause or contribute to fatigue. […] The management of liver-related complications in PBC is the same as the management for patients with advanced liver disease related to other types of chronic liver diseases. […] The panel recommends that the first line treatment for PBC patients should be UDCA. For PBC patients who are intolerant to UDCA, OCA monotherapy should be started. […] For patients who are non-responsive to the appropriate dose of UDCA at 6-12 months, OCA should be added to the regimen.

• #16 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. […] Approximately 20-30% of PBC patients have incomplete biochemical response with UDCA. Those with incomplete response were found to have poorer outcomes than those with complete response. […] Pruritus is often refractory to medical therapy and significantly impacts the patient’s quality of life. Antihistamines are the first-line agents to relieve pruritus in the early stages and are the first line medication for patients with mild-to-moderate pruritus. Use with caution in patients with cirrhosis and signs of encephalopathy because antihistamines can further depress neurologic function.

• #17 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. […] Management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. […] Disease management focuses on initiation of UDCA for all patients and risk stratification based on baseline and on-treatment factors, including in particular the response to treatment. […] We recommend oral UDCA at 1315mg/kg/day is used as the first-line pharmacotherapy in all patients with PBC. If tolerated, treatment should usually be life-long. […] We recommend all patients should be evaluated for the presence of symptoms, particularly fatigue and itch. Clinicians should recognise that severity of symptoms does not correlate with stage of disease.

• #18 Primary Biliary Cholangitis (PBC) – Cirrhosis Care

  https://cirrhosiscare.ca/patients/cirrhosis/causes/pbc/

  PBC is a chronic liver disease: a lasting condition that can be controlled in most people but not cured. The prognosis for people with PBC has improved over the last two decades due to earlier diagnosis and improved treatment. Early access to treatment can delay the progression of the disease. Treatment improves outcomes for people with PBC, and sometimes, it can even improve outcomes so that they are similar to people without the disease. […] A drug called ursodeoxycholic acid (also called UDCA or URSO) mimics a bile acid your body produces naturally. URSO can improve liver function and slow the onset of fibrosis (scar tissue) in the liver. In turn, this can delay or eliminate the possibility of liver failure and the need for a liver transplant. 80 to 90% of people with PBC on URSO manage to avoid the need for a liver transplant after 10 years. Patients usually take this drug indefinitely. Fortunately, its well tolerated and has minimal side effects.

• #19 Primary biliary cirrhosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/primary-biliary-cirrhosis

  Primary biliary cholangitis (previously known as primary biliary cirrhosis) is characterised by the chronic inflammation and scarring of the bile ducts within the liver. […] There is no cure, but treatment can help slow the progression of disease and ease associated symptoms. […] The disease is usually diagnosed later in life, between the ages of 35 and 60 years. There is no cure, but treatment can slow the progression of the disease and alleviate symptoms. […] Fortunately, most people with PBC don’t ever experience this degree of cirrhosis. […] As a result, this disorder is now called primary biliary cholangitis, which refers to inflammation of the bile ducts. The older term primary biliary cirrhosis is no longer recommended. […] There is no cure for PBC, but treatment can help slow the progression of disease and associated symptoms. Options include: medication, particularly ursodeoxycholic acid (Ursofalk) for patients who do not respond, obeticholic acid (not available yet in Australia) and fibrates can be considered. […] Regular weight bearing exercise (such as walking or weight training) and calcium or vitamin D supplementation to reduce the risk of osteoporosis. […] Liver transplant, in severe cases.

• #20 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

  PBC is a disease that destroys these bile ducts over time. […] If you have PBC, you’re at higher risk for other health issues like: […] Our liver experts: […] Develop advanced therapies for people with PBC. […] Doctors can’t cure PBC. But they can treat you to help stop or slow further liver damage. […] Healthy habits are especially vital when you have PBC. […] Your doctor might prescribe certain medications to help slow liver damage from PBC. […] Over time, PBC may damage the liver so much that you experience liver failure or end-stage liver disease.

• #21 Primary Biliary Cirrhosis | UCSF Department of Surgery

  https://surgery.ucsf.edu/condition/primary-biliary-cirrhosis

  Health care providers prescribe ursodiol (Actigall, Urso) to treat primary biliary cirrhosis. Early treatment with this medication reduces the likelihood of needing a liver transplant and improves survival. […] A health care provider may consider a liver transplant when cirrhosis leads to liver failure or treatment for complications is ineffective.

• #22 Primary Biliary Cholangitis (PBC) – Cirrhosis Care

  https://cirrhosiscare.ca/patients/cirrhosis/causes/pbc/

  PBC is a chronic liver disease: a lasting condition that can be controlled in most people but not cured. The prognosis for people with PBC has improved over the last two decades due to earlier diagnosis and improved treatment. Early access to treatment can delay the progression of the disease. Treatment improves outcomes for people with PBC, and sometimes, it can even improve outcomes so that they are similar to people without the disease. […] A drug called ursodeoxycholic acid (also called UDCA or URSO) mimics a bile acid your body produces naturally. URSO can improve liver function and slow the onset of fibrosis (scar tissue) in the liver. In turn, this can delay or eliminate the possibility of liver failure and the need for a liver transplant. 80 to 90% of people with PBC on URSO manage to avoid the need for a liver transplant after 10 years. Patients usually take this drug indefinitely. Fortunately, its well tolerated and has minimal side effects.

• #23 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. […] Approximately 20-30% of PBC patients have incomplete biochemical response with UDCA. Those with incomplete response were found to have poorer outcomes than those with complete response. […] Pruritus is often refractory to medical therapy and significantly impacts the patient’s quality of life. Antihistamines are the first-line agents to relieve pruritus in the early stages and are the first line medication for patients with mild-to-moderate pruritus. Use with caution in patients with cirrhosis and signs of encephalopathy because antihistamines can further depress neurologic function.

• #24 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). […] Major quality of life (QoL)-impacting symptoms of PBC, including pruritus and fatigue, are demonstrated to be independent of disease severity. There is evidence confirming that these symptoms negatively impact a number of aspects, including emotional status, ability to work, and social life, for some patients. […] 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.

• #25 Primary Biliary Cholangitis & Non-Alcoholic Steatohepatitis – ICER

  https://icer.org/assessment/primary-biliary-cholangitis-non-alcoholic-steatohepatitis-2016/

  Obeticholic acid (OCA) is a novel bile acid analogue that has shown positive effects on biochemical markers of liver function in phase II trials and was approved by the FDA for treatment of primary biliary cholangitis (PBC) after failure of ursodeoxycholic Acid (UDCA). Primary biliary cholangitis (PBC), which has until recently been called primary biliary cirrhosis, is a rare, chronic, progressive autoimmune liver disease that affects mainly middle-aged women. In the US, up to 130,000 individuals may have PBC. […] A majority of the Council voted that for patients with PBC who do not have an adequate response to usual care with ursodeoxycholic acid (UDCA), the evidence demonstrates that OCA provides a net health benefit. […] ICERs Evidence Report reviewing the clinical effectiveness and value of obeticholic acid for treatment of primary biliary cholangitis. This report was the subject of the July 15, 2016 meeting of the New England Comparative Effectiveness Public Advisory Council, during which the independent Council deliberated and voted on evidence presented in the report and discussed the implications of their votes with a policy roundtable of experts in the field to develop recommendations to guide policy and practice. […] Patients with PBC have very limited options when it comes to treatments. Were pleased that our report was able to shed light on the potential health benefit of OCA for people with PBC who arent benefiting from currently available treatments.

• #26

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

  Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis. It’s a type of liver disease caused by damage to the bile ducts in the liver. PBC permanently damages the liver as tissue is replaced with scar tissue. As more scar tissue develops, the structure and function of the liver are affected. […] Treatment for PBC focuses on: […] Reducing symptoms. […] Preventing and treating the complications of the disease. […] Preventing other conditions that may cause more liver damage. […] Two medicines can be used to treat PBC. They are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA). Both medicines help move bile out of the liver into the small intestine. And OCA decreases the amount of bile acids made by the liver. […] 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.

• #27 Primary biliary cirrhosis | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/primary-biliary-cirrhosis

  Primary biliary cholangitis (previously known as primary biliary cirrhosis) is characterised by the chronic inflammation and scarring of the bile ducts within the liver. […] There is no cure, but treatment can help slow the progression of disease and ease associated symptoms. […] The disease is usually diagnosed later in life, between the ages of 35 and 60 years. There is no cure, but treatment can slow the progression of the disease and alleviate symptoms. […] Fortunately, most people with PBC don’t ever experience this degree of cirrhosis. […] As a result, this disorder is now called primary biliary cholangitis, which refers to inflammation of the bile ducts. The older term primary biliary cirrhosis is no longer recommended. […] There is no cure for PBC, but treatment can help slow the progression of disease and associated symptoms. Options include: medication, particularly ursodeoxycholic acid (Ursofalk) for patients who do not respond, obeticholic acid (not available yet in Australia) and fibrates can be considered. […] Regular weight bearing exercise (such as walking or weight training) and calcium or vitamin D supplementation to reduce the risk of osteoporosis. […] Liver transplant, in severe cases.

• #28 Extrahepatic Manifestations of Primary Biliary Cholangitis

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl16365

  Pruritus is the second most common symptom in PBC and affects 40% to 80% of patients. […] There is decreased bile acid secretion in PBC, so vitamin deficiencies can be present as there is increased risk of malabsorption of fat-soluble vitamins. […] Given the propensity for patients with PBC to develop extrahepatic manifestations, almost all of them being autoimmune, awareness and close screening is imperative. A summary of screening recommendations can be seen in Table 4. Upon initial diagnosis, a thorough review of systems should be obtained. It would also be prudent to conduct targeted serologic screening including a thyroid panel, anti-Ro/SSA, anti-La/SSB and anti-centromere antibody. […] The patient care team should include practitioners in rheumatology, endocrinology, pulmonology and cardiology when indicated. Patients should follow regularly with their primary care physicians. As some of these extrahepatic manifestations can lead to diseases with a poor prognosis, vigilant screening and close follow-up will lead to prompt identification and treatment.

• #29 Primary Biliary Cholangitis (PBC) – Cirrhosis Care

  https://cirrhosiscare.ca/patients/cirrhosis/causes/pbc/

  To help relieve itching (pruritus), a common symptom, your doctor may prescribe various medications such as cholestyramine, antihistamines, or other drugs such as naltrexone or rifampicin. For people with extreme itching, doctors will occasionally prescribe ultraviolet light therapy. […] Your doctor will also discuss other treatments for you to consider. These can include calcium and vitamin D to preserve your bone health. It may also involve other fat-soluble vitamin supplements and treatments for managing symptoms such as dry eyes, mouth, or vagina.

• #30 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. […] Approximately 20-30% of PBC patients have incomplete biochemical response with UDCA. Those with incomplete response were found to have poorer outcomes than those with complete response. […] Pruritus is often refractory to medical therapy and significantly impacts the patient’s quality of life. Antihistamines are the first-line agents to relieve pruritus in the early stages and are the first line medication for patients with mild-to-moderate pruritus. Use with caution in patients with cirrhosis and signs of encephalopathy because antihistamines can further depress neurologic function.

• #31

  https://www.nuhs.edu.sg/patient-care/find-a-condition/primary-biliary-cirrhosis-pbc

  Cholestyramine (also known as Questran) may be prescribed by your doctor to help ease the itch. […] A liver transplant is usually only recommended if other treatments are no longer helpful and your life is threatened by end stage liver disease. It is a major operation and you will need to plan it carefully with your medical team, family and friends. […] However, liver transplantation works well for people with PBC.

• #32

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  Given the variability of the natural history among PBC patients, individual predictive tools may be useful in patient counseling and management planning, including referral for liver transplantation. […] In this context, early diagnosis and treatment of PBC is essential. Historically, a number of criteria were required to establish the diagnosis of PBC. […] It is important to note that liver biopsy is not required for PBC, unless specific antibodies are absent or co-existence of NASH or autoimmune hepatitis (AIH) is suspected. […] Pruritus occurs in 20-70% of patients with PBC and is not associated with the degree of laboratory abnormalities, disease duration, or histologic severity. […] Treatment of pruritus is important in PBC patients in order to improve mood, quality of sleep, and social functioning. AASLD and EASL guidelines recommend a number of non-pharmacologic interventions to manage pruritus. […] Recent advances in the understanding of bile acid physiology and pathophysiology of cholestatic pruritus have led to the development of novel therapies to treat pruritus in PBC.

• #33

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

• #34

  https://www.nuh.com.sg/health-resources/diseases-and-conditions/primary-biliary-cirrhosis-pbc

  A medication called ursodeoxycholic acid has proven effective for some patients. Although early studies did not show any direct impact on survival rates, new evidence suggests that URSO may help to keep people alive for a longer period of time and delay liver transplantation. […] For people who have advanced PBC, however, a liver transplant is their only option. […] Itching skin: cholestyramine (also known as Questran) may be prescribed to help ease the itch. Taken orally, cholestyramine works by preventing re-absorption of the chemicals that cause itching. […] If cholestyramine does not help, a hospital specialist may try other medicines. Itching is made worse by dry skin, thus it is very important to apply plenty of moisturiser. […] A liver transplant is usually only recommended if other treatments are no longer effective and one’s life is threatened by end-stage liver disease.

• #35 Primary Biliary Cholangitis Pain: 5 Tips To Help | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-pain

  Primary biliary cholangitis (PBC), previously primary biliary cirrhosis, is an autoimmune disease that affects the liver. However, symptoms of PBC can occur throughout the body, including various types of pain. This pain can be a direct result of PBC or associated with other conditions PBC can cause, such as edema (severe swelling) and osteoporosis (weak bones). […] If you’re diagnosed with PBC and experience pain, it may or may not be directly related to your condition. It is essential to inform your health care provider to determine the root cause and find the most effective treatment. Prescription medications such as obeticholic acid (Ocaliva) and ursodeoxycholic acid (UDCA, sold as Ursodiol) are direct treatment options for PBC. Additionally, various over-the-counter and prescription medications can help manage conditions associated with PBC.

• #36 Extrahepatic Manifestations of Primary Biliary Cholangitis

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl16365

  Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogrens syndrome, thyroid dysfunction and systemic sclerosis. […] Due to the high prevalence of extrahepatic diseases in patients with PBC, it is important to complete a thorough medical history at the time of diagnosis. Prompt recognition of extrahepatic disease can lead to improved patient outcomes and quality of life. […] Fatigue is the most common clinical manifestation of PBC. It is present in up to 80% of patients and fluctuates independently of disease activity or stage.

• #37 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/

  UDCA has been shown to improve biochemistry and long-term outcomes with a reduced need for liver transplantation and death. […] Biochemical liver tests can help healthcare professionals identify the best therapy option for patients with PBC. However, biochemistry will not necessarily be tackling symptom burden. […] In patients with PBC presenting moderatesevere symptoms, impacts include pruritus, fatigue, cognitive problems, social impairment, and emotional dysfunction. […] Cholestatic pruritus is one of the major symptoms of PBC, with around half of all patients experiencing this to some degree at any given time. […] Addressing pruritus symptoms is essential, highlighted Jones, because if you can roll the itch back, you can start to roll back several other difficult symptoms. […] Fatigue as a symptom of PBC can also greatly impact a patients QoL, with one study showing this occurred in 68.0% of patients.

• #38

  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. […] Ursodeoxycholic acid (UDCA) is the main treatment for PBC. […] Once you start taking UDCA, it’s likely you’ll need to take it for the rest of your life. […] People who may need OCA will first need to be assessed by a specialist team with experience in treating PBC. […] Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC. […] No medicines are currently available to specifically treat fatigue associated with PBC. […] A liver transplant may be recommended if it’s thought the liver damage may eventually put your life at risk. […] Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue. […] But having a liver transplant does not always completely cure PBC.

• #39

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  In patients with PBC and fatigue, it is important to exclude or identify and manage potential causes of fatigue such as anemia, depression, sleep disorders, hypothyroidism, and medications that can cause or contribute to fatigue. […] The management of liver-related complications in PBC is the same as the management for patients with advanced liver disease related to other types of chronic liver diseases. […] The panel recommends that the first line treatment for PBC patients should be UDCA. For PBC patients who are intolerant to UDCA, OCA monotherapy should be started. […] For patients who are non-responsive to the appropriate dose of UDCA at 6-12 months, OCA should be added to the regimen.

• #40 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

• #41 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459209/

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. Prolonged hepatic cholestasis subsequently causes cirrhosis and portal hypertension. This activity reviews the evaluation and treatment of PBC and the importance of the interprofessional team in recognizing and managing patients with this condition. […] The management of primary biliary cholangitis is best done through an interprofessional team approach. The disorder has no cure and carries high morbidity and mortality. Treatment aims to slow disease progression and improve the quality of life. Besides the physicians, the nurse, mental health counselor, pharmacist, and physical therapist are critical in managing these patients. The nurse should educate the patient on the management of itching and the use of moisturizers. The dietitian should educate the patient on a healthy diet to prevent osteoporosis and deficiency of fat-soluble vitamins. The pharmacist should educate the patient on the Sicca syndrome and avoid dry eyes and mouth. Because of extreme fatigue, the patient should enroll in a physical therapy program or physical activity. Finally, the patient should see a mental health counselor as the disorder can result in premature death. The patient should be urged to join a support group and closely follow up with the healthcare provider.

• #42

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  Primary biliary cholangitis is a slowly progressive immune-mediated cholestatic disease that causes a destruction of the intrahepatic bile ducts and may lead to cirrhosis of the liver, end-stage liver disease, and the need for liver transplantation. […] Advanced practice registered nurses need to understand the pathophysiology, clinical presentation, diagnostic approaches, disease and symptom management, and priority nursing assessment and care in patients with this rare disease to differentiate it from primary sclerosing cholangitis, autoimmune hepatitis, obstructed bile duct lesions, drug-induced cholestasis, cholestasis in pregnancy, cholangiocarcinoma, hepatic malignancy, and peptic ulcer disease. […] The nurse should consider the following questions to assess the underlying cause of fatigue: Is the fatigue related to (1) the PBC disease process, (2) comorbidities, (3) medication side effects, and (4) the patient’s mental health?

• #43 Primary Biliary Cholangitis (PBC) – Cirrhosis Care

  https://cirrhosiscare.ca/patients/cirrhosis/causes/pbc/

  To help relieve itching (pruritus), a common symptom, your doctor may prescribe various medications such as cholestyramine, antihistamines, or other drugs such as naltrexone or rifampicin. For people with extreme itching, doctors will occasionally prescribe ultraviolet light therapy. […] Your doctor will also discuss other treatments for you to consider. These can include calcium and vitamin D to preserve your bone health. It may also involve other fat-soluble vitamin supplements and treatments for managing symptoms such as dry eyes, mouth, or vagina.

• #44

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

• #45

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

  People who have end-stage PBC may need a liver transplant. But PBC can come back after a liver transplant. […] There are things you can do at home. For example, if your doctor prescribed medicines, take them as instructed. Don’t drink alcohol. If your eyes feel dry, try eyedrops. Sugar-free gum or candy can help increase saliva if your mouth is dry. And stay up to date on your vaccines.

• #46 Primary Biliary Cholangitis | CommonSpirit Health

  https://www.commonspirit.org/conditions-treatments/primary-biliary-cholangitis

  Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis and is a type of liver disease caused by damage to the bile ducts in the liver. […] Treatment for primary biliary cholangitis (PBC) is difficult, because the immune system appears to be involved. […] Two medicines, ursodeoxycholic acid (UDCA) and obeticholic acid (OCA), can be used to treat PBC. […] PBC can also cause dry eyes and mouth. The best way to help dry eyes is to use eye drops (artificial tears) when your eyes feel dry. […] Stop drinking alcohol completely. Doing this is important, even if alcohol wasn’t the cause of PBC. […] Because people with PBC have a high risk of getting osteoporosis, you should have periodic bone density scans. […] There are things you can do at home. For example, if your doctor prescribed medicines, take them as instructed. Don’t drink alcohol. If your eyes feel dry, try eyedrops. Sugar-free gum or candy can help increase saliva if your mouth is dry. And stay up to date on your vaccines.

• #47 Extrahepatic Manifestations of Primary Biliary Cholangitis

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl16365

  Pruritus is the second most common symptom in PBC and affects 40% to 80% of patients. […] There is decreased bile acid secretion in PBC, so vitamin deficiencies can be present as there is increased risk of malabsorption of fat-soluble vitamins. […] Given the propensity for patients with PBC to develop extrahepatic manifestations, almost all of them being autoimmune, awareness and close screening is imperative. A summary of screening recommendations can be seen in Table 4. Upon initial diagnosis, a thorough review of systems should be obtained. It would also be prudent to conduct targeted serologic screening including a thyroid panel, anti-Ro/SSA, anti-La/SSB and anti-centromere antibody. […] The patient care team should include practitioners in rheumatology, endocrinology, pulmonology and cardiology when indicated. Patients should follow regularly with their primary care physicians. As some of these extrahepatic manifestations can lead to diseases with a poor prognosis, vigilant screening and close follow-up will lead to prompt identification and treatment.

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

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

  Patients should be managed with calcium and vitamin D supplementation, along with weight-bearing exercise. For those with bone density loss, therapy with such medications as bisphosphonates is recommended. […] 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).

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

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

  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. […] People with PBC are at risk for nutritional deficiencies due to poor absorption of nutrients. You may receive vitamin and mineral supplements to replace missing nutrients, guided by our expert dietitians. […] Your doctors will monitor your bone density because people with PBC are also at risk for osteoporosis. […] If your PBC becomes so advanced that it cannot be effectively managed with other treatments, you may need a liver transplant. […] Portal hypertension (increased pressure in the portal vein, the major vein transporting blood from your stomach to your liver and other digestive organs) is a serious complication of PBC. Some people develop varices (enlarged veins in the stomach or esophagus).

• #50

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

  Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis. It’s a type of liver disease caused by damage to the bile ducts in the liver. PBC permanently damages the liver as tissue is replaced with scar tissue. As more scar tissue develops, the structure and function of the liver are affected. […] Treatment for PBC focuses on: […] Reducing symptoms. […] Preventing and treating the complications of the disease. […] Preventing other conditions that may cause more liver damage. […] Two medicines can be used to treat PBC. They are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA). Both medicines help move bile out of the liver into the small intestine. And OCA decreases the amount of bile acids made by the liver. […] 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.

• #51 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 is no cure for Primary Biliary Cholangitis (PBC), however, there are medications that can help slow disease progression and manage symptoms. […] Liver transplantation is considered when medical treatment no longer sufficiently controls the disease. […] 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.

• #52 Primary Biliary Cholangitis (PBC) | Cigna

  https://www.cigna.com/knowledge-center/hw/medical-topics/primary-biliary-cholangitis-aa89995spec

  Primary biliary cholangitis (PBC) used to be called primary biliary cirrhosis. It’s a type of liver disease caused by damage to the bile ducts in the liver. PBC permanently damages the liver as tissue is replaced with scar tissue. As more scar tissue develops, the structure and function of the liver are affected. […] Treatment for PBC focuses on: […] Reducing symptoms. […] 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. If your eyes feel dry, try eyedrops. Sugar-free gum or candy can help increase saliva if your mouth is dry. And stay up to date on your vaccines.

• #53 Primary Biliary Cholangitis Pain: 5 Tips To Help | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-pain

  PBC can make it difficult to absorb fat-soluble vitamins like vitamins A and D, essential for bone strength. As a result, people with PBC are more likely to develop osteoporosis, which reduces bone density and can cause muscle and bone pain, particularly in the back. It may even lead to fractures with minimal or no trauma. […] Advanced PBC can cause edema, a buildup of fluid and swelling in the feet and ankles. This excess fluid can stretch the skin, causing pain and discomfort. […] A healthy liver produces liver enzymes that clear bilirubin, a yellow waste product from old red blood cells, from your body through bile. When the liver cannot process all of the bilirubin, it circulates in the blood, causing jaundice (yellowing of the skin and eyes). PBC can lead to a bilirubin buildup, resulting in jaundice and symptoms such as belly pain and itching. […] Dietary changes can help manage jaundice and other PBC symptoms. The American Liver Foundation recommends increasing intake of fruits, vegetables, complex carbohydrates, and whole grains. The nonprofit also suggests moderating your intake of lean meats and avoiding too much sugar and sodium.

• #54 Primary Biliary Cholangitis: Prognosis, Stages, and Symptoms

  https://www.healthline.com/health/primary-biliary-cirrhosis

  Your doctor can also prescribe medications to treat symptoms and complications of PBC, such as: […] Youll also need to avoid alcohol since it can further damage your liver. […] If you become deficient in fat-soluble vitamins, you can take supplements to replace them. Taking calcium and vitamin D can help keep your bones strong. […] Better treatments have improved the outlook for people with PBC in recent years. People who respond to treatment earlier in the course of the illness are more likely to have a typical life expectancy. […] To have the best possible outlook, follow the treatment your doctor prescribes. Stay healthy with diet, exercise, and not smoking or drinking alcohol.

• #55 Primary Biliary Cholangitis: Symptoms & Causes

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

  Since people with Primary Biliary Cholangitis (PBC) are at a higher risk for osteoporosis, calcium and vitamin D are usually prescribed. […] 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). […] Keep in mind that Primary Biliary Cholangitis (PBC) usually advances slowly over a period of years. […] Women are nine times more likely than men to develop Primary Biliary Cholangitis (PBC), meaning that women make up about 90% of Primary Biliary Cholangitis (PBC) cases. […] The disease most often develops during middle age and is usually diagnosed in people between the ages of 35 to 60 years.

• #56 Primary Biliary Cholangitis (PBC)

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

  If the treatment with UDCA is not sufficient to stabilise the disease, additional treatment is wanted to further improve the prognosis. […] Your hepatologist is responsible for the clinical management of your PBC, and the coordination with other specialists in conjunction with your family doctor. […] How PBC is monitored is dependent on the severity of the disease. […] There is no special diet for PBC. The recommendation will be to maintain a healthy, well-balanced diet, as for the general population. […] If you are of childbearing age, you can discuss your wish to become pregnant with your doctor. […] PBC is a rare disease and, as such, not every doctor may have sufficient experience to treat patients with PBC.

• #57

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

• #58 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

• #59 Primary biliary cholangitis – Knowledge @ AMBOSS

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

  Provide treatment for hypertension. […] Optimize bone health, e.g., consider supplementing calcium and vitamin D if needed. […] Monitoring for disease progression includes obtaining liver and cholestatic enzymes every 3-6 months to assess for disease progression, therapy adherence, and development of PBC-AIH overlap. […] Screen for complications of cholestasis, including osteoporosis and fat-soluble vitamin deficiency in patients with jaundice and/or bilirubin 2.0 mg/dL. […] Screen for complications of cirrhosis, including HCC screening and screening for esophageal varices.

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

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

  Patients should be managed with calcium and vitamin D supplementation, along with weight-bearing exercise. For those with bone density loss, therapy with such medications as bisphosphonates is recommended. […] 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).

• #61

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  Given the variability of the natural history among PBC patients, individual predictive tools may be useful in patient counseling and management planning, including referral for liver transplantation. […] In this context, early diagnosis and treatment of PBC is essential. Historically, a number of criteria were required to establish the diagnosis of PBC. […] It is important to note that liver biopsy is not required for PBC, unless specific antibodies are absent or co-existence of NASH or autoimmune hepatitis (AIH) is suspected. […] Pruritus occurs in 20-70% of patients with PBC and is not associated with the degree of laboratory abnormalities, disease duration, or histologic severity. […] Treatment of pruritus is important in PBC patients in order to improve mood, quality of sleep, and social functioning. AASLD and EASL guidelines recommend a number of non-pharmacologic interventions to manage pruritus. […] Recent advances in the understanding of bile acid physiology and pathophysiology of cholestatic pruritus have led to the development of novel therapies to treat pruritus in PBC.

• #62

  https://www.nuh.com.sg/health-resources/diseases-and-conditions/primary-biliary-cirrhosis-pbc

  Liver transplantation can provide positive outcomes for those with PBC. It is possible to develop PBC in the new liver, but it may take up to 15 years before the disease progresses significantly. […] Once diagnosed, the patient normally visits their liver clinic at regular intervals to monitor the progress of the condition.

• #63 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

• #64

  https://journals.lww.com/ajg/fulltext/2019/01000/diagnosis_and_management_of_primary_biliary.13.aspx

  Given the variability of the natural history among PBC patients, individual predictive tools may be useful in patient counseling and management planning, including referral for liver transplantation. […] In this context, early diagnosis and treatment of PBC is essential. Historically, a number of criteria were required to establish the diagnosis of PBC. […] It is important to note that liver biopsy is not required for PBC, unless specific antibodies are absent or co-existence of NASH or autoimmune hepatitis (AIH) is suspected. […] Pruritus occurs in 20-70% of patients with PBC and is not associated with the degree of laboratory abnormalities, disease duration, or histologic severity. […] Treatment of pruritus is important in PBC patients in order to improve mood, quality of sleep, and social functioning. AASLD and EASL guidelines recommend a number of non-pharmacologic interventions to manage pruritus. […] Recent advances in the understanding of bile acid physiology and pathophysiology of cholestatic pruritus have led to the development of novel therapies to treat pruritus in PBC.

• #65 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. […] Management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. […] Disease management focuses on initiation of UDCA for all patients and risk stratification based on baseline and on-treatment factors, including in particular the response to treatment. […] We recommend oral UDCA at 1315mg/kg/day is used as the first-line pharmacotherapy in all patients with PBC. If tolerated, treatment should usually be life-long. […] We recommend all patients should be evaluated for the presence of symptoms, particularly fatigue and itch. Clinicians should recognise that severity of symptoms does not correlate with stage of disease.

• #66 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/

  A patient-centred approach to PBC requires symptom assessment and treatment rather than focusing only on biochemical indices, particularly given that symptom burden does not correlate with disease severity. Pruritis and fatigue are two common PBC symptoms that significantly impact patient QoL and are under-recognised by physicians.

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

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

  Patients should be managed with calcium and vitamin D supplementation, along with weight-bearing exercise. For those with bone density loss, therapy with such medications as bisphosphonates is recommended. […] 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).

• #68 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

  PBC is a disease that destroys these bile ducts over time. […] If you have PBC, you’re at higher risk for other health issues like: […] Our liver experts: […] Develop advanced therapies for people with PBC. […] Doctors can’t cure PBC. But they can treat you to help stop or slow further liver damage. […] Healthy habits are especially vital when you have PBC. […] Your doctor might prescribe certain medications to help slow liver damage from PBC. […] Over time, PBC may damage the liver so much that you experience liver failure or end-stage liver disease.

• #69 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  We recommend that patients with PBC should be offered the chance to seek support from patient support groups. […] We recommend that clinicians caring for patients with PBC should consider introducing clinical audit tools to document and improve the quality of care delivered to patients. […] We recommend that all patients with PBC should have a risk assessment for osteoporosis. Treatment and follow-up should be according to national guidelines. […] We recommend that liver transplantation can be an effective treatment for advanced PBC and eligibility should be assessed in line with national guidelines. […] We recommend that pruritus refractory to all medical therapy can be an indication for liver transplantation in highly selected patients regardless of disease stage.

• #70

  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. […] Ursodeoxycholic acid (UDCA) is the main treatment for PBC. […] Once you start taking UDCA, it’s likely you’ll need to take it for the rest of your life. […] People who may need OCA will first need to be assessed by a specialist team with experience in treating PBC. […] Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC. […] No medicines are currently available to specifically treat fatigue associated with PBC. […] A liver transplant may be recommended if it’s thought the liver damage may eventually put your life at risk. […] Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue. […] But having a liver transplant does not always completely cure PBC.

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

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

  Patients should be managed with calcium and vitamin D supplementation, along with weight-bearing exercise. For those with bone density loss, therapy with such medications as bisphosphonates is recommended. […] 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).

• #72

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

  People who have end-stage PBC may need a liver transplant. But PBC can come back after a liver transplant. […] There are things you can do at home. For example, if your doctor prescribed medicines, take them as instructed. Don’t drink alcohol. If your eyes feel dry, try eyedrops. Sugar-free gum or candy can help increase saliva if your mouth is dry. And stay up to date on your vaccines.

• #73

  https://www.nuh.com.sg/health-resources/diseases-and-conditions/primary-biliary-cirrhosis-pbc

  Liver transplantation can provide positive outcomes for those with PBC. It is possible to develop PBC in the new liver, but it may take up to 15 years before the disease progresses significantly. […] Once diagnosed, the patient normally visits their liver clinic at regular intervals to monitor the progress of the condition.

• #74

  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. […] Ursodeoxycholic acid (UDCA) is the main treatment for PBC. […] Once you start taking UDCA, it’s likely you’ll need to take it for the rest of your life. […] People who may need OCA will first need to be assessed by a specialist team with experience in treating PBC. […] Colestyramine (previously called cholestyramine) is a medicine widely used to treat the itchiness associated with PBC. […] No medicines are currently available to specifically treat fatigue associated with PBC. […] A liver transplant may be recommended if it’s thought the liver damage may eventually put your life at risk. […] Having a liver transplant will cure the itchiness and other symptoms, but you may still have fatigue. […] But having a liver transplant does not always completely cure PBC.

• #75

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

  Primary biliary cholangitis (PBC, often referred to as primary biliary cirrhosis) is a type of liver disease that can get gradually worse over time. Without treatment, it may eventually lead to liver failure. […] PBC is a progressive condition, which means the damage to the liver can steadily get worse over time. […] Without treatment, the liver can become so badly damaged that it no longer works properly. This is known as liver failure and can be fatal. […] Current treatments such as ursodeoxycholic acid and obeticholic acid can help slow down liver damage caused by PBC. […] Other medicines can help relieve the itchiness associated with PBC. Occasionally, if the liver is severely damaged, a liver transplant may be needed.

• #76

  https://www.nuh.com.sg/health-resources/diseases-and-conditions/primary-biliary-cirrhosis-pbc

  Liver transplantation can provide positive outcomes for those with PBC. It is possible to develop PBC in the new liver, but it may take up to 15 years before the disease progresses significantly. […] Once diagnosed, the patient normally visits their liver clinic at regular intervals to monitor the progress of the condition.

• #77 Primary biliary cholangitis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/primary-biliary-cholangitis

  If you have weak or thinning bones, called osteoporosis, you may be prescribed medicines or supplements, such as calcium and vitamin D, to reduce bone loss and improve bone density. Exercise such as walking and using light weights most days of the week can help increase your bone density. […] Living with an ongoing liver disease with no cure can be frustrating. Fatigue alone can have a large impact on your quality of life. […] The more you understand about primary biliary cholangitis, the more active you can be in your own care.

• #78 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  We recommend that patients with PBC should be offered the chance to seek support from patient support groups. […] We recommend that clinicians caring for patients with PBC should consider introducing clinical audit tools to document and improve the quality of care delivered to patients. […] We recommend that all patients with PBC should have a risk assessment for osteoporosis. Treatment and follow-up should be according to national guidelines. […] We recommend that liver transplantation can be an effective treatment for advanced PBC and eligibility should be assessed in line with national guidelines. […] We recommend that pruritus refractory to all medical therapy can be an indication for liver transplantation in highly selected patients regardless of disease stage.

• #79 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459209/

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. Prolonged hepatic cholestasis subsequently causes cirrhosis and portal hypertension. This activity reviews the evaluation and treatment of PBC and the importance of the interprofessional team in recognizing and managing patients with this condition. […] The management of primary biliary cholangitis is best done through an interprofessional team approach. The disorder has no cure and carries high morbidity and mortality. Treatment aims to slow disease progression and improve the quality of life. Besides the physicians, the nurse, mental health counselor, pharmacist, and physical therapist are critical in managing these patients. The nurse should educate the patient on the management of itching and the use of moisturizers. The dietitian should educate the patient on a healthy diet to prevent osteoporosis and deficiency of fat-soluble vitamins. The pharmacist should educate the patient on the Sicca syndrome and avoid dry eyes and mouth. Because of extreme fatigue, the patient should enroll in a physical therapy program or physical activity. Finally, the patient should see a mental health counselor as the disorder can result in premature death. The patient should be urged to join a support group and closely follow up with the healthcare provider.

• #80 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

• #81 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/

  UDCA has been shown to improve biochemistry and long-term outcomes with a reduced need for liver transplantation and death. […] Biochemical liver tests can help healthcare professionals identify the best therapy option for patients with PBC. However, biochemistry will not necessarily be tackling symptom burden. […] In patients with PBC presenting moderatesevere symptoms, impacts include pruritus, fatigue, cognitive problems, social impairment, and emotional dysfunction. […] Cholestatic pruritus is one of the major symptoms of PBC, with around half of all patients experiencing this to some degree at any given time. […] Addressing pruritus symptoms is essential, highlighted Jones, because if you can roll the itch back, you can start to roll back several other difficult symptoms. […] Fatigue as a symptom of PBC can also greatly impact a patients QoL, with one study showing this occurred in 68.0% of patients.

• #82 Primary biliary cholangitis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/primary-biliary-cholangitis

  If you have weak or thinning bones, called osteoporosis, you may be prescribed medicines or supplements, such as calcium and vitamin D, to reduce bone loss and improve bone density. Exercise such as walking and using light weights most days of the week can help increase your bone density. […] Living with an ongoing liver disease with no cure can be frustrating. Fatigue alone can have a large impact on your quality of life. […] The more you understand about primary biliary cholangitis, the more active you can be in your own care.

• #83 Primary Biliary Cholangitis (PBC)

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

  If the treatment with UDCA is not sufficient to stabilise the disease, additional treatment is wanted to further improve the prognosis. […] Your hepatologist is responsible for the clinical management of your PBC, and the coordination with other specialists in conjunction with your family doctor. […] How PBC is monitored is dependent on the severity of the disease. […] There is no special diet for PBC. The recommendation will be to maintain a healthy, well-balanced diet, as for the general population. […] If you are of childbearing age, you can discuss your wish to become pregnant with your doctor. […] PBC is a rare disease and, as such, not every doctor may have sufficient experience to treat patients with PBC.

• #84

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

  People who have end-stage PBC may need a liver transplant. But PBC can come back after a liver transplant. […] There are things you can do at home. For example, if your doctor prescribed medicines, take them as instructed. Don’t drink alcohol. If your eyes feel dry, try eyedrops. Sugar-free gum or candy can help increase saliva if your mouth is dry. And stay up to date on your vaccines.

• #85 Primary Biliary Cirrhosis Symptoms and Treatment | UI Health | UI Health

  https://hospital.uillinois.edu/primary-and-specialty-care/hepatology-liver-disease/areas-of-expertise/primary-biliary-cirrhosis

  Primary biliary cirrhosis – also known as primary biliary cholangitis – is a type of liver disease that causes damage to the bile ducts in the liver. Over time, the disease fully destroys the bile ducts, which are responsible for carrying bile from the liver to the small intestine. […] It is important to seek medical treatment when symptoms or complications first emerge, since in many cases, treatment with medication can slow liver damage. The condition generally progresses slowly, so early intervention can improve outcomes. […] If you’re diagnosed with the condition, a number of treatment options – including medications, supplementation with fat-soluble vitamins, or surgery – are available to alleviate symptoms and slow or delay the disease progress. In cases of liver failure, a liver transplant may be necessary. […] To prevent further liver damage, your physician will likely also recommend lifestyle changes, including quitting smoking, not drinking alcohol, eating a well-balanced diet, and maintaining a healthy weight.

• #86 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  For patients with the chronic progressive liver disease primary biliary cholangitis (PBC), personalised treatment is key to both preserving liver function and retaining health-related quality of life (HRQoL). […] According to symposium chair Gideon Hirshfield, such symptoms necessitate a patient-centric approach to PBC symptom management. […] Both symptoms need to be discussed and assessed at every visit to a healthcare professional (HCP), with the need for treatment guided by the patient. […] Christie concluded by discussing how we need to inform, educate, and empower patients to be involved in their own care journey. […] Hirschfield stressed how this patient-centric approach, where symptoms are readily addressed and managed in addition to starting disease-modifying therapy, could make a difference to HRQoL.

• #87 Primary biliary cholangitis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/primary-biliary-cholangitis

  If you have weak or thinning bones, called osteoporosis, you may be prescribed medicines or supplements, such as calcium and vitamin D, to reduce bone loss and improve bone density. Exercise such as walking and using light weights most days of the week can help increase your bone density. […] Living with an ongoing liver disease with no cure can be frustrating. Fatigue alone can have a large impact on your quality of life. […] The more you understand about primary biliary cholangitis, the more active you can be in your own care.

• #88

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

• #89 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  Primary biliary cholangitis (formerly known as primary biliary cirrhosis, PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. […] Management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. […] Disease management focuses on initiation of UDCA for all patients and risk stratification based on baseline and on-treatment factors, including in particular the response to treatment. […] We recommend oral UDCA at 1315mg/kg/day is used as the first-line pharmacotherapy in all patients with PBC. If tolerated, treatment should usually be life-long. […] We recommend all patients should be evaluated for the presence of symptoms, particularly fatigue and itch. Clinicians should recognise that severity of symptoms does not correlate with stage of disease.

• #90 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines | Gut

  https://gut.bmj.com/content/67/9/1568

  We recommend that patients with PBC should be offered the chance to seek support from patient support groups. […] We recommend that clinicians caring for patients with PBC should consider introducing clinical audit tools to document and improve the quality of care delivered to patients. […] We recommend that all patients with PBC should have a risk assessment for osteoporosis. Treatment and follow-up should be according to national guidelines. […] We recommend that liver transplantation can be an effective treatment for advanced PBC and eligibility should be assessed in line with national guidelines. […] We recommend that pruritus refractory to all medical therapy can be an indication for liver transplantation in highly selected patients regardless of disease stage.

• #91

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

• #92 Extrahepatic Manifestations of Primary Biliary Cholangitis

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl16365

  Pruritus is the second most common symptom in PBC and affects 40% to 80% of patients. […] There is decreased bile acid secretion in PBC, so vitamin deficiencies can be present as there is increased risk of malabsorption of fat-soluble vitamins. […] Given the propensity for patients with PBC to develop extrahepatic manifestations, almost all of them being autoimmune, awareness and close screening is imperative. A summary of screening recommendations can be seen in Table 4. Upon initial diagnosis, a thorough review of systems should be obtained. It would also be prudent to conduct targeted serologic screening including a thyroid panel, anti-Ro/SSA, anti-La/SSB and anti-centromere antibody. […] The patient care team should include practitioners in rheumatology, endocrinology, pulmonology and cardiology when indicated. Patients should follow regularly with their primary care physicians. As some of these extrahepatic manifestations can lead to diseases with a poor prognosis, vigilant screening and close follow-up will lead to prompt identification and treatment.

• #93 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. […] Your healthcare team includes specialists who diagnose and treat PBC, including hepatologists (liver doctors), gastroenterologists, interventional endoscopists, liver surgeons, physician assistants, nurses, registered dietitians, social workers, and others. All of the healthcare professionals you may need are available to you through one medical center. […] 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.

• #94 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459209/

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. Prolonged hepatic cholestasis subsequently causes cirrhosis and portal hypertension. This activity reviews the evaluation and treatment of PBC and the importance of the interprofessional team in recognizing and managing patients with this condition. […] The management of primary biliary cholangitis is best done through an interprofessional team approach. The disorder has no cure and carries high morbidity and mortality. Treatment aims to slow disease progression and improve the quality of life. Besides the physicians, the nurse, mental health counselor, pharmacist, and physical therapist are critical in managing these patients. The nurse should educate the patient on the management of itching and the use of moisturizers. The dietitian should educate the patient on a healthy diet to prevent osteoporosis and deficiency of fat-soluble vitamins. The pharmacist should educate the patient on the Sicca syndrome and avoid dry eyes and mouth. Because of extreme fatigue, the patient should enroll in a physical therapy program or physical activity. Finally, the patient should see a mental health counselor as the disorder can result in premature death. The patient should be urged to join a support group and closely follow up with the healthcare provider.

• #95 Primary Biliary Cholangitis | PBC Treatment – UChicago Medicine

  https://www.uchicagomedicine.org/conditions-services/liver-diseases-hepatology/primary-biliary-cholangitis-pbc

  Providing Specialized Care for Primary Biliary Cholangitis. University of Chicago Medicine physicians collaborate closely to form a multidisciplinary team that is highly skilled in diagnosing and treating primary biliary cholangitis. Your treatment plan will be developed by specialists and designed to fit your specific needs. […] Currently, there is no known treatment to cure primary biliary cholangitis. However, certain medications can help slow its progression, and your healthcare provider can treat you for the symptoms and complications associated with the disease. […] Although primary biliary cholangitis cant be cured, its progression can be slowed with ursodeoxycholic acid (UCDA) and obeticholic acid, medications that can help improve the bile flow in your liver. Your health care provider can also treat your PBC symptoms; cholestyramine, colestipol hydrochloride, antihistamines, rifampin and naltrexone are all medicines that may be used to treat the itching caused by PBC. […] If primary biliary cholangitis eventually causes your liver to fail, your doctor may recommend a liver transplant. Liver transplants can cure primary biliary cholangitis, although PBC may recur in the new liver in a small number of patients.

• #96

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

• #97 Primary Biliary Cirrhosis – Causes, Symptoms and Treatments

  https://nowpatient.com/conditions/primary-biliary-cirrhosis

  Regular monitoring and follow-up care are crucial for individuals with primary biliary cholangitis. This may involve regular blood tests to assess liver function, monitor medication effectiveness, and detect any potential complications. […] Early detection, proper management, lifestyle changes, and adherence to treatment can significantly slow disease progression and improve outcomes for individuals with PBC.

• #98

  https://www.nursingcenter.com/journalarticle?Article_ID=5510691&Journal_ID=54035&Issue_ID=5509793

  The first-line therapy for pruritis is bile acid sequestrants, such as cholestyramine, which not only lower cholesterol in hypercholesterolemia but also metabolize excessive bile from the blood and improve pruritis in 90% of patients. […] Nutritional deficits from malabsorption are best addressed by a dietician in collaboration with the nurse. […] Patients with PBC with sicca syndrome should stay well-hydrated to provide mucous membranes of the eyes and mouth with moisture to prevent infection. […] The nurse should assess for ascites and peripheral edema. Fluid overload correlates with an increase in body weight and abdominal girth and a decrease in serum albumin and proteins. […] The interprofessional team should establish target outcomes that reflect PBC disease management and symptom control. Outcomes should include the following: (1) liver enzymes and bilirubin will normalize, (2) antibody titers will decrease or be negative, (3) histological improvement through repeat liver biopsy will be achieved, (4) physical and psychological symptoms will be controlled, (5) optimal nutritional status will be maintained, and (6) optimal activity level will be maintained.

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

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

  Primary Biliary Cholangitis (PBC) is a chronic liver disease that primarily affects the small bile ducts within the liver. […] If left untreated, PBC can lead to serious complications such as: Cirrhosis: Severe scarring of the liver tissue, which impairs liver function […] While there is no cure for PBC, several treatments can help manage the disease and slow its progression: Lifestyle Changes: A healthy Mediterranean type-diet low in processed foods, coffee, vitamin D, regular exercise, and avoiding/limiting alcohol can help support liver health and prevent complications. […] Topical treatment- menthol aqueous cream can sooth itching and it is important to keep nails short […] In severe cases where the liver is significantly damaged, a liver transplant might be the best option. […] Early diagnosis, appropriate medical management, and lifestyle changes can make a significant difference in managing the disease and maintaining liver health.

• #100 Primary Biliary Cholangitis (PBC) – HealthyWomen

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

  Your health care provider may conduct these tests to help you avoid complications: Blood tests may be used to monitor for deficiencies in fat-soluble vitamins. […] Maintaining a healthy lifestyle can help people with PBC feel better, as well as relieve or prevent some symptoms associated with the disease. […] Many people lead normal lives for years without symptoms, depending on how early the diagnosis is made. […] PBC is an autoimmune disease that cannot be prevented or cured, but there is treatment available to slow disease progression.

• #101 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/

  A patient-centred approach to PBC requires symptom assessment and treatment rather than focusing only on biochemical indices, particularly given that symptom burden does not correlate with disease severity. Pruritis and fatigue are two common PBC symptoms that significantly impact patient QoL and are under-recognised by physicians.

• #102 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

• #103 Exploring life and parenthood with Primary Biliary Cholangitis  – Global

  https://www.ipsen.com/rare-diseases/exploring-life-and-parenthood-with-primary-biliary-cholangitis/

  For many of those living with PBC, the challenges do not stop with the symptoms, and it is not uncommon to face delays in getting a diagnosis. Not only this but once you have been diagnosed, available treatments do not always effectively manage the disease progression or address the symptoms that impact on quality of life. […] Trying to be the best parent you can be while living with a condition like PBC is a momentous ask, and Sabrina’s story resonates with other individuals out there who are navigating life with a long-term disease, whilst also raising their children. […] We are dedicated to developing new transformative treatment options that both manage the disease progression and address the effects of symptoms of rare liver disease.

• #104 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

• #105 Re-imagining Primary Biliary Cholangitis Care: Patient-Centric, Utilising Biochemistry, Controlling Symptoms – European Medical Journal

  https://www.emjreviews.com/hepatology/symposium/re-imagining-primary-biliary-cholangitis-care-patient-centric-utilising-biochemistry-controlling-symptoms-j070124/

  Risk stratification is important, said Londoo, because during follow-up, it will help us to make decisions to improve the treatment and care of patients. […] Jones concluded that it is important that pruritus is discussed at every visit so that patients know that what they are experiencing may be related to PBC. […] Jones also suggested to emphasise how it was important for patients to maintain social structure and ensure that they left the consultation feeling positive, even if this is just about them taking ownership of their problems. […] Fatigue should be regularly measured in all patients with PBC to properly assess disease-related burden. […] In particular, we can help them to understand the symptoms and to live the best life they can with it; its about helping people to cope.

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