Materiały źródłowe

• #1 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. […] Symptoms of PBC include the following: Fatigue (65% of patients): The first reported symptom, Pruritus (55%), Right upper quadrant discomfort (8-17%). […] As the disease advances, the following signs may be noted: Hepatomegaly (25%), Hyperpigmentation (25%), Splenomegaly (15%), Jaundice (10%), Xanthomas and Xanthelasmas (10%): In late stages of the disease, Sicca syndrome (50-75%): Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth).

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

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

  Primary biliary cholangitis is a chronic and progressive condition that causes inflammation and, eventually, the destruction of the bile ducts that run through your liver. Without working bile ducts, bile backs up in your liver, causing liver damage. This can lead to cirrhosis of the liver. But medication can delay and sometimes prevent it. […] 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.

• #3 Rare and Serious Liver Diseases – Intercept Pharmaceuticals

  https://www.interceptpharma.com/our-focus/pbc/

  Primary biliary cholangitis (PBC) is a rare liver disease that is caused by an autoimmune reaction. The autoimmune reaction damages bile ducts in the liver. Bile ducts are tube-like structures that carry bile from the liver to the intestine to help with the digestion of food. […] PBC is progressive, which means that the damage gets worse over time. Starting with inflammation, the damage can cause fibrosis, and then cirrhosis. In some cases, cirrhosis can lead to liver failure. […] The rate of damage caused by PBC varies in different people. If left untreated, PBC can get significantly worse in 2 years, making it important to get an early diagnosis to start treatment as quickly as possible. […] Once someone is diagnosed with PBC, it is important to talk to a doctor about getting regular liver function tests to track the progression of the disease.

• #4 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. […] Eventually this can lead to cirrhosis and its associated complications, as scar tissue replaces healthy liver tissue and liver function becomes increasingly impaired. […] PBC may progress slowly and many people do not have symptoms, particularly in the early stages of the disease. The most common initial symptoms are fatigue and itching of the skin (pruritus). Other symptoms may include: abdominal pain, darkening of the skin, small yellow or white bumps under the skin (xanthomas) or around the eyes (xanthelasmas). […] As the disease progresses, symptoms of cirrhosis can develop including: yellowing of the skin (jaundice), swelling of the legs and feet (edema), enlarged abdomen from fluid accumulation (ascites), internal bleeding in the upper stomach and esophagus from enlarged veins (varices).

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

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

  Many people with PBC have no symptoms in the early stages. As the disease progresses, signs of biliary disease begin to appear. The earliest and most common symptoms of people with PBC are: Fatigue (65%). Itchy skin (55%). […] However, not everyone discovers PBC in time to treat it in the early stages. And some people simply have a more aggressive form of the disease. Higher levels of fatigue and higher levels of bilirubin in your blood predict a faster progression to liver failure. If you reach this stage, you’ll need a liver transplant to survive. But for those with PBC who have successful liver transplants, the prognosis is excellent. […] It takes an average of 15 to 20 years for PBC to progress to the terminal stage. The first stage, when you test positive for PBC but you don’t yet have symptoms, can last a long time. About half of people will begin having symptoms in the next five to 10 years. Once you have symptoms, the average life expectancy is about 10 years. For those who have successful liver transplants, the 10-year survival rate is 65%.

• #6

  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 does not always cause symptoms, but some people may experience: bone and joint aches, fatigue (extreme tiredness), itchy skin, dry eyes and mouth, pain or discomfort in the upper right side of their tummy. […] PBC is a progressive condition, which means the damage to the liver can steadily get worse over time. […] The rate at which PBC progresses varies between individuals. Sometimes, it can take decades. […] 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.

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

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

  Many people with PBC have no symptoms in the early stages. As the disease progresses, signs of biliary disease begin to appear. The earliest and most common symptoms of people with PBC are: Fatigue (65%). Itchy skin (55%). […] However, not everyone discovers PBC in time to treat it in the early stages. And some people simply have a more aggressive form of the disease. Higher levels of fatigue and higher levels of bilirubin in your blood predict a faster progression to liver failure. If you reach this stage, you’ll need a liver transplant to survive. But for those with PBC who have successful liver transplants, the prognosis is excellent. […] It takes an average of 15 to 20 years for PBC to progress to the terminal stage. The first stage, when you test positive for PBC but you don’t yet have symptoms, can last a long time. About half of people will begin having symptoms in the next five to 10 years. Once you have symptoms, the average life expectancy is about 10 years. For those who have successful liver transplants, the 10-year survival rate is 65%.

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

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

  Usually, PBC progresses to terminal stages over 15 to 20 years, although the rate of progression varies. PBC may not diminish quality of life for many years. Patients who present without symptoms tend to develop symptoms over 2 to 7 years but may not do so for 10 to 15 years. Once symptoms develop, median life expectancy is 10 years. Predictors of rapid progression include the following: Rapid worsening of symptoms, Advanced histologic changes, Older patient age, Presence of edema, Presence of associated autoimmune disorders, Abnormalities in bilirubin, albumin, prothrombin time, or international normalized ratio (INR). […] The prognosis is ominous when pruritus disappears, xanthomas shrink, jaundice develops, and serum cholesterol decreases.

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

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

  Many people with PBC have no symptoms in the early stages. As the disease progresses, signs of biliary disease begin to appear. The earliest and most common symptoms of people with PBC are: Fatigue (65%). Itchy skin (55%). […] However, not everyone discovers PBC in time to treat it in the early stages. And some people simply have a more aggressive form of the disease. Higher levels of fatigue and higher levels of bilirubin in your blood predict a faster progression to liver failure. If you reach this stage, you’ll need a liver transplant to survive. But for those with PBC who have successful liver transplants, the prognosis is excellent. […] It takes an average of 15 to 20 years for PBC to progress to the terminal stage. The first stage, when you test positive for PBC but you don’t yet have symptoms, can last a long time. About half of people will begin having symptoms in the next five to 10 years. Once you have symptoms, the average life expectancy is about 10 years. For those who have successful liver transplants, the 10-year survival rate is 65%.

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

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

  Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disorder characterized by progressive cholestatic that, if untreated, can progress to liver fibrosis, cirrhosis and liver decompensation requiring liver transplant. […] PBC is a progressive hepatic disease and the early stages of the disease manifest with fatigue, pruritus, right upper quadrant abdominal discomfort and jaundice. […] The introduction of ursodeoxycholic acid (UDCA) in the treatment of PBC patients has changed the disease course, improving transplant-free survival rates to up to 60% of patients, thereby reducing mortality. Currently, life expectancy in PBC patients under UDCA therapy is similar to non-PBC patients. […] PBC is considered an organ-specific autoimmune disorder and, along with the autoimmune hepatitis (AIH) and the primary sclerosing cholangitis (PSC), is classified as an autoimmune liver disease.

• #11 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  At times, your primary biliary cholangitis (PBC) might feel like a runaway train. Previously known as primary biliary cirrhosis, PBC is a progressive liver disease that continues to worsen over time. […] Doctors divide PBC into four stages based on severity of symptoms. Progression to a higher stage may be slowed with proper treatment. […] In this article, well discuss the four stages of PBC and their symptoms as the disease progresses. Its important to reach out to your doctor if you think your PBC is reaching a new stage. The sooner you start treatment, the better chance you have of slowing disease progression. […] PBC affects every person differently, but there are general characteristics and symptoms of each stage. […] Early-stage PBC might not cause any symptoms, so it can be difficult to tell what stage you have.

• #12 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  In stage 1 PBC, these small bile ducts in your liver start becoming inflamed. This is known as portal inflammation. […] Around 95 percent of people with PBC make immune system proteins known as autoantibodies. […] As PBC progresses to the second stage, inflammation begins spreading from the portal area to nearby tissues. This inflammation can damage and kill liver cells. […] Most people begin experiencing symptoms with stage 3 PBC. This is because the liver starts malfunctioning when more scar tissue builds up. […] The symptoms of stage 3 PBC are usually associated with cholestasis. You may experience pruritus (itchy skin) due to the buildup of bile acids (bile salts) in your bloodstream. […] The fourth stage of PBC is associated with cirrhosis severe liver scarring. Your liver has fewer functional bile ducts and cant efficiently transport bile to your digestive system.

• #13 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  In stage 1 PBC, these small bile ducts in your liver start becoming inflamed. This is known as portal inflammation. […] Around 95 percent of people with PBC make immune system proteins known as autoantibodies. […] As PBC progresses to the second stage, inflammation begins spreading from the portal area to nearby tissues. This inflammation can damage and kill liver cells. […] Most people begin experiencing symptoms with stage 3 PBC. This is because the liver starts malfunctioning when more scar tissue builds up. […] The symptoms of stage 3 PBC are usually associated with cholestasis. You may experience pruritus (itchy skin) due to the buildup of bile acids (bile salts) in your bloodstream. […] The fourth stage of PBC is associated with cirrhosis severe liver scarring. Your liver has fewer functional bile ducts and cant efficiently transport bile to your digestive system.

• #14 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  In stage 1 PBC, these small bile ducts in your liver start becoming inflamed. This is known as portal inflammation. […] Around 95 percent of people with PBC make immune system proteins known as autoantibodies. […] As PBC progresses to the second stage, inflammation begins spreading from the portal area to nearby tissues. This inflammation can damage and kill liver cells. […] Most people begin experiencing symptoms with stage 3 PBC. This is because the liver starts malfunctioning when more scar tissue builds up. […] The symptoms of stage 3 PBC are usually associated with cholestasis. You may experience pruritus (itchy skin) due to the buildup of bile acids (bile salts) in your bloodstream. […] The fourth stage of PBC is associated with cirrhosis severe liver scarring. Your liver has fewer functional bile ducts and cant efficiently transport bile to your digestive system.

• #15 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  In stage 1 PBC, these small bile ducts in your liver start becoming inflamed. This is known as portal inflammation. […] Around 95 percent of people with PBC make immune system proteins known as autoantibodies. […] As PBC progresses to the second stage, inflammation begins spreading from the portal area to nearby tissues. This inflammation can damage and kill liver cells. […] Most people begin experiencing symptoms with stage 3 PBC. This is because the liver starts malfunctioning when more scar tissue builds up. […] The symptoms of stage 3 PBC are usually associated with cholestasis. You may experience pruritus (itchy skin) due to the buildup of bile acids (bile salts) in your bloodstream. […] The fourth stage of PBC is associated with cirrhosis severe liver scarring. Your liver has fewer functional bile ducts and cant efficiently transport bile to your digestive system.

• #16 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  Other PBC stage 4 symptoms are due to disease complications. […] Without enough bile, your digestive system will have a harder time breaking down and absorbing fats. […] As your PBC progresses, your doctor will likely change your care plan. […] Unfortunately, theres no cure or way to reverse PBC. […] The U.S. Food and Drug Administration (FDA) has approved three PBC treatments. […] Be sure to talk to your doctor if your PBC symptoms change over time. It may be a sign your disease is progressing.

• #17 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #18 Symptoms & Causes of Primary Biliary Cholangitis (Primary Biliary Cirrhosis) – NIDDK

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

  The most common symptoms of primary biliary cholangitis are feeling tired and having itchy skin. […] As the disease gets worse, symptoms may include darkening of skin color, fatty deposits that appear as yellow bumps on the skin, called xanthomas, and symptoms of cirrhosis, such as edema, jaundice, and weight loss. […] Many people have no symptoms when they are first diagnosed with primary biliary cholangitis. Doctors diagnose about 60% of people with primary biliary cholangitis before symptoms begin. People with primary biliary cholangitis and no symptoms are identified through blood tests. Some people do not have symptoms for years after they have been diagnosed with primary biliary cholangitis.

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

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

  Many people with PBC have no symptoms in the early stages. As the disease progresses, signs of biliary disease begin to appear. The earliest and most common symptoms of people with PBC are: Fatigue (65%). Itchy skin (55%). […] However, not everyone discovers PBC in time to treat it in the early stages. And some people simply have a more aggressive form of the disease. Higher levels of fatigue and higher levels of bilirubin in your blood predict a faster progression to liver failure. If you reach this stage, you’ll need a liver transplant to survive. But for those with PBC who have successful liver transplants, the prognosis is excellent. […] It takes an average of 15 to 20 years for PBC to progress to the terminal stage. The first stage, when you test positive for PBC but you don’t yet have symptoms, can last a long time. About half of people will begin having symptoms in the next five to 10 years. Once you have symptoms, the average life expectancy is about 10 years. For those who have successful liver transplants, the 10-year survival rate is 65%.

• #20 Rare and Serious Liver Diseases – Intercept Pharmaceuticals

  https://www.interceptpharma.com/our-focus/pbc/

  The most common symptoms of PBC are pruritus, or itching, and fatigue. Some people with PBC can also experience dry eyes and mouth. For people with more advanced PBC, serious liver damage can cause jaundice, or a yellowing of the skin and eyes. […] Many people do not experience any symptoms at the beginning of their PBC. The only sign that they have PBC is that abnormalities appear in their liver function test results.

• #21

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

  Most people are only diagnosed with PBC after having a routine blood test for another reason. Some people have symptoms early on and some may develop them later. […] People who have symptoms may experience: bone or joint aches, fatigue this is a common symptom (but not always caused by PBC) and can have a significant impact on your daily activities, itchy skin this can be widespread or only affect a single area; it may be worse at night, when in contact with fabrics, when warm, or during pregnancy, dry eyes and mouth, feeling very sleepy during the day, pain or discomfort in the upper right side of the tummy. […] Symptoms can range from mild to severe, and this is not always related to the degree of liver damage you have. […] Some people with PBC have severe symptoms but their liver is not severely damaged, while others may have significant liver damage but no symptoms or only mild ones.

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

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

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease caused by damage to bile ducts in the liver. Damage to the bile ducts allows bile to build up in the liver. Over time, the accumulated bile damages the liver. It can lead to permanent scarring and cirrhosis. […] After their diagnosis, a person doesnt typically experience symptoms until 2 to 4.2 years later. However, some people may not have symptoms for over 17 years. […] The first symptoms are often fatigue, dry mouth, and dry eyes along with itchy skin. […] Later symptoms can include: belly pain, darkening of the skin, nausea, appetite loss, weight loss, dry eyes and mouth, small yellow or white bumps under the skin (xanthomas) or eyes (xanthelasmas), joint, muscle, or bone pain, yellowing of the skin and eyes (jaundice), swollen belly from fluid buildup, swelling in the legs and ankles (edema), diarrhea, fractures caused by weakened bones.

• #23 Symptoms of primary biliary cholangitis – British Liver Trust

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

  In the early stages of primary biliary cholangitis (PBC), you may not have any symptoms at all. This is very common, as PBC is such a slowly developing condition. […] You may feel a bit run down, or under the weather. Its this type of vague symptom that can take people to the GP for the first time. […] You may also have vague symptoms such as: feeling very tired, sleeping more than usual, having itchy skin for no apparent reason, pain or discomfort in the tummy (abdomen). […] As many as 4 out of 5 people with PBC are troubled by itching at some point. Developing itching doesnt mean your PBC is getting worse. In fact itching sometimes gets better as the disease continues. […] Some people develop yellow spots on the eyelids or around their eyes. These are harmless in themselves. They are caused by fatty cholesterol deposits under the skin. You may also have dry eyes and a dry mouth.

• #24

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

  Most people are only diagnosed with PBC after having a routine blood test for another reason. Some people have symptoms early on and some may develop them later. […] People who have symptoms may experience: bone or joint aches, fatigue this is a common symptom (but not always caused by PBC) and can have a significant impact on your daily activities, itchy skin this can be widespread or only affect a single area; it may be worse at night, when in contact with fabrics, when warm, or during pregnancy, dry eyes and mouth, feeling very sleepy during the day, pain or discomfort in the upper right side of the tummy. […] Symptoms can range from mild to severe, and this is not always related to the degree of liver damage you have. […] Some people with PBC have severe symptoms but their liver is not severely damaged, while others may have significant liver damage but no symptoms or only mild ones.

• #25 Primary Biliary Cholangitis: 10 Symptoms To Know | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-symptoms-to-know

  Itchy skin also known as pruritus is common in people living with PBC. […] If you have PBC, you may develop pruritus at any point. […] Between 47 percent and 73 percent of people with PBC experience dry eyes and mouth. […] A complication of PBC called portal hypertension can cause fluid to collect in different parts of your body. […] Abdominal pain is common in people with PBC. […] People with PBC have an increased risk of osteoporosis, a disease that weakens your bones. […] Many people with PBC also report pain in their bones, muscles, or joints. […] If you have PBC, you may notice changes in the color or appearance of your skin. […] Between 75 percent and 95 percent of people with PBC also have high cholesterol. […] In advanced PBC, you may have difficulty absorbing fats, which is called fat malabsorption. […] Hypothyroidism (an underactive thyroid) is more common in people with PBC than in the general population.

• #26 Primary Biliary Cholangitis: 10 Symptoms To Know | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-symptoms-to-know

  Itchy skin also known as pruritus is common in people living with PBC. […] If you have PBC, you may develop pruritus at any point. […] Between 47 percent and 73 percent of people with PBC experience dry eyes and mouth. […] A complication of PBC called portal hypertension can cause fluid to collect in different parts of your body. […] Abdominal pain is common in people with PBC. […] People with PBC have an increased risk of osteoporosis, a disease that weakens your bones. […] Many people with PBC also report pain in their bones, muscles, or joints. […] If you have PBC, you may notice changes in the color or appearance of your skin. […] Between 75 percent and 95 percent of people with PBC also have high cholesterol. […] In advanced PBC, you may have difficulty absorbing fats, which is called fat malabsorption. […] Hypothyroidism (an underactive thyroid) is more common in people with PBC than in the general population.

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

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

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease caused by damage to bile ducts in the liver. Damage to the bile ducts allows bile to build up in the liver. Over time, the accumulated bile damages the liver. It can lead to permanent scarring and cirrhosis. […] After their diagnosis, a person doesnt typically experience symptoms until 2 to 4.2 years later. However, some people may not have symptoms for over 17 years. […] The first symptoms are often fatigue, dry mouth, and dry eyes along with itchy skin. […] Later symptoms can include: belly pain, darkening of the skin, nausea, appetite loss, weight loss, dry eyes and mouth, small yellow or white bumps under the skin (xanthomas) or eyes (xanthelasmas), joint, muscle, or bone pain, yellowing of the skin and eyes (jaundice), swollen belly from fluid buildup, swelling in the legs and ankles (edema), diarrhea, fractures caused by weakened bones.

• #28

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

  Most people are only diagnosed with PBC after having a routine blood test for another reason. Some people have symptoms early on and some may develop them later. […] People who have symptoms may experience: bone or joint aches, fatigue this is a common symptom (but not always caused by PBC) and can have a significant impact on your daily activities, itchy skin this can be widespread or only affect a single area; it may be worse at night, when in contact with fabrics, when warm, or during pregnancy, dry eyes and mouth, feeling very sleepy during the day, pain or discomfort in the upper right side of the tummy. […] Symptoms can range from mild to severe, and this is not always related to the degree of liver damage you have. […] Some people with PBC have severe symptoms but their liver is not severely damaged, while others may have significant liver damage but no symptoms or only mild ones.

• #29 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. […] Symptoms of PBC include the following: Fatigue (65% of patients): The first reported symptom, Pruritus (55%), Right upper quadrant discomfort (8-17%). […] As the disease advances, the following signs may be noted: Hepatomegaly (25%), Hyperpigmentation (25%), Splenomegaly (15%), Jaundice (10%), Xanthomas and Xanthelasmas (10%): In late stages of the disease, Sicca syndrome (50-75%): Xerophthalmia (ie, dry eyes), xerostomia (ie, dry mouth).

• #30

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

  Most people are only diagnosed with PBC after having a routine blood test for another reason. Some people have symptoms early on and some may develop them later. […] People who have symptoms may experience: bone or joint aches, fatigue this is a common symptom (but not always caused by PBC) and can have a significant impact on your daily activities, itchy skin this can be widespread or only affect a single area; it may be worse at night, when in contact with fabrics, when warm, or during pregnancy, dry eyes and mouth, feeling very sleepy during the day, pain or discomfort in the upper right side of the tummy. […] Symptoms can range from mild to severe, and this is not always related to the degree of liver damage you have. […] Some people with PBC have severe symptoms but their liver is not severely damaged, while others may have significant liver damage but no symptoms or only mild ones.

• #31 Primary Biliary Cholangitis: 10 Symptoms To Know | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-symptoms-to-know

  Itchy skin also known as pruritus is common in people living with PBC. […] If you have PBC, you may develop pruritus at any point. […] Between 47 percent and 73 percent of people with PBC experience dry eyes and mouth. […] A complication of PBC called portal hypertension can cause fluid to collect in different parts of your body. […] Abdominal pain is common in people with PBC. […] People with PBC have an increased risk of osteoporosis, a disease that weakens your bones. […] Many people with PBC also report pain in their bones, muscles, or joints. […] If you have PBC, you may notice changes in the color or appearance of your skin. […] Between 75 percent and 95 percent of people with PBC also have high cholesterol. […] In advanced PBC, you may have difficulty absorbing fats, which is called fat malabsorption. […] Hypothyroidism (an underactive thyroid) is more common in people with PBC than in the general population.

• #32

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

  Most people are only diagnosed with PBC after having a routine blood test for another reason. Some people have symptoms early on and some may develop them later. […] People who have symptoms may experience: bone or joint aches, fatigue this is a common symptom (but not always caused by PBC) and can have a significant impact on your daily activities, itchy skin this can be widespread or only affect a single area; it may be worse at night, when in contact with fabrics, when warm, or during pregnancy, dry eyes and mouth, feeling very sleepy during the day, pain or discomfort in the upper right side of the tummy. […] Symptoms can range from mild to severe, and this is not always related to the degree of liver damage you have. […] Some people with PBC have severe symptoms but their liver is not severely damaged, while others may have significant liver damage but no symptoms or only mild ones.

• #33 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. […] Disease course is frequently accompanied by symptoms that can be burdensome for patients, and management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. […] The presence of antimitochondrial antibodies (1 in 40) or highly PBC-specific antinuclear antibodies, in the appropriate context of cholestatic liver biochemistry, without alternative explanation, is usually sufficient for confidently reaching the diagnosis of PBC. […] 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.

• #34

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

• #35 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #36

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

• #37 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. […] Eventually this can lead to cirrhosis and its associated complications, as scar tissue replaces healthy liver tissue and liver function becomes increasingly impaired. […] PBC may progress slowly and many people do not have symptoms, particularly in the early stages of the disease. The most common initial symptoms are fatigue and itching of the skin (pruritus). Other symptoms may include: abdominal pain, darkening of the skin, small yellow or white bumps under the skin (xanthomas) or around the eyes (xanthelasmas). […] As the disease progresses, symptoms of cirrhosis can develop including: yellowing of the skin (jaundice), swelling of the legs and feet (edema), enlarged abdomen from fluid accumulation (ascites), internal bleeding in the upper stomach and esophagus from enlarged veins (varices).

• #38

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

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

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

  Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease caused by damage to bile ducts in the liver. Damage to the bile ducts allows bile to build up in the liver. Over time, the accumulated bile damages the liver. It can lead to permanent scarring and cirrhosis. […] After their diagnosis, a person doesnt typically experience symptoms until 2 to 4.2 years later. However, some people may not have symptoms for over 17 years. […] The first symptoms are often fatigue, dry mouth, and dry eyes along with itchy skin. […] Later symptoms can include: belly pain, darkening of the skin, nausea, appetite loss, weight loss, dry eyes and mouth, small yellow or white bumps under the skin (xanthomas) or eyes (xanthelasmas), joint, muscle, or bone pain, yellowing of the skin and eyes (jaundice), swollen belly from fluid buildup, swelling in the legs and ankles (edema), diarrhea, fractures caused by weakened bones.

• #40

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

• #41 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  PBC may progress to cirrhosis, this implies that there is significant scarring (fibrosis) in the liver with the formation of nodules. […] Many people who have cirrhosis will remain well with no complications or side-effects; this is termed Compensated Cirrhosis. […] The term Decompensated Cirrhosis means that the patient is developing complications of the cirrhosis. […] Patients with cirrhosis, of whatever cause, are at higher risk of developing cancer of the liver (hepatocellular carcinoma but also known as hepatoma). […] When the liver fails to clear the toxins from the bowel, the toxins will enter the circulation and may cause hepatic encephalopathy (HE). […] Ascites is the build-up of fluid in the abdomen. […] There are many causes of ankle swelling (pedal oedema). […] Varices are dilated veins which can develop as a consequence of advanced scarring and cirrhosis, whatever the cause of the cirrhosis. […] Jaundice is the result of a build-up of bilirubin in the blood and leads to a yellow colour in the eyes, and skin. […] Portal hypertension can be a consequence of cirrhosis.

• #42 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #43 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. […] Eventually this can lead to cirrhosis and its associated complications, as scar tissue replaces healthy liver tissue and liver function becomes increasingly impaired. […] PBC may progress slowly and many people do not have symptoms, particularly in the early stages of the disease. The most common initial symptoms are fatigue and itching of the skin (pruritus). Other symptoms may include: abdominal pain, darkening of the skin, small yellow or white bumps under the skin (xanthomas) or around the eyes (xanthelasmas). […] As the disease progresses, symptoms of cirrhosis can develop including: yellowing of the skin (jaundice), swelling of the legs and feet (edema), enlarged abdomen from fluid accumulation (ascites), internal bleeding in the upper stomach and esophagus from enlarged veins (varices).

• #44 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #45 Predicting outcome in primary biliary cirrhosis | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-predicting-outcome-in-primary-biliary-S1665268119308385

  Once symptoms appear, survival of initially asymptomatic patients is comparable with survival of patients who initially presented with symptoms. […] The ability to reliably predict outcome in patients with PBC is critically important in clinical management and an essential requirement for patient counselling and timing of diagnostic procedures and therapeutic interventions. […] Male gender and advanced disease are the most frequently reported risk factors for HCC in PBC.

• #46 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #47 Primary Biliary Cholangitis: Symptoms & Causes

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

  Thinning of the bones (osteoporosis) leading to fractures is another complication of Primary Biliary Cholangitis (PBC). […] While this is more common in late stages of the disease, it can occur earlier as well. […] Keep in mind that Primary Biliary Cholangitis (PBC) usually advances slowly over a period of years. Many people lead normal lives for years without symptoms, depending on how early the diagnosis is made. And while there is no cure, people are having good results slowing disease progression and living longer without complications by adhering to their medication regimen and maintaining a healthy lifestyle.

• #48 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #49 Primary Biliary Cholangitis: 10 Symptoms To Know | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-symptoms-to-know

  Itchy skin also known as pruritus is common in people living with PBC. […] If you have PBC, you may develop pruritus at any point. […] Between 47 percent and 73 percent of people with PBC experience dry eyes and mouth. […] A complication of PBC called portal hypertension can cause fluid to collect in different parts of your body. […] Abdominal pain is common in people with PBC. […] People with PBC have an increased risk of osteoporosis, a disease that weakens your bones. […] Many people with PBC also report pain in their bones, muscles, or joints. […] If you have PBC, you may notice changes in the color or appearance of your skin. […] Between 75 percent and 95 percent of people with PBC also have high cholesterol. […] In advanced PBC, you may have difficulty absorbing fats, which is called fat malabsorption. […] Hypothyroidism (an underactive thyroid) is more common in people with PBC than in the general population.

• #50 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #51 PBC Stages and Symptoms: Understanding the Progression | myPBCteam

  https://www.mypbcteam.com/resources/pbc-stages-and-symptoms-understanding-the-progression

  Other PBC stage 4 symptoms are due to disease complications. […] Without enough bile, your digestive system will have a harder time breaking down and absorbing fats. […] As your PBC progresses, your doctor will likely change your care plan. […] Unfortunately, theres no cure or way to reverse PBC. […] The U.S. Food and Drug Administration (FDA) has approved three PBC treatments. […] Be sure to talk to your doctor if your PBC symptoms change over time. It may be a sign your disease is progressing.

• #52 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #53 Primary Biliary Cholangitis: 10 Symptoms To Know | myPBCteam

  https://www.mypbcteam.com/resources/primary-biliary-cholangitis-symptoms-to-know

  Itchy skin also known as pruritus is common in people living with PBC. […] If you have PBC, you may develop pruritus at any point. […] Between 47 percent and 73 percent of people with PBC experience dry eyes and mouth. […] A complication of PBC called portal hypertension can cause fluid to collect in different parts of your body. […] Abdominal pain is common in people with PBC. […] People with PBC have an increased risk of osteoporosis, a disease that weakens your bones. […] Many people with PBC also report pain in their bones, muscles, or joints. […] If you have PBC, you may notice changes in the color or appearance of your skin. […] Between 75 percent and 95 percent of people with PBC also have high cholesterol. […] In advanced PBC, you may have difficulty absorbing fats, which is called fat malabsorption. […] Hypothyroidism (an underactive thyroid) is more common in people with PBC than in the general population.

• #54 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #55 Primary Biliary Cirrhosis – Shore Gastroenterology Associates

  https://shoregastro.com/conditions-and-diseases/primary-biliary-cirrhosis/

  PBC is a slow and progressive disease, and you may not experience symptoms for years. […] If you do experience symptoms, some of the most common symptoms include nausea and vomiting, darkening or yellowing of the skin, unexplained weight loss, sudden fatigue, intense itching, a swollen stomach, edema (swelling of the legs and ankles), diarrhea, and joint pain. […] As PBC is a progressive disease, it’s best to discover it in its early stages to optimize life expectancy. […] Primary biliary cholangitis can be present for years before symptoms occur. […] However, if the disease isn’t discovered until later or treatment isn’t initiated, the average life expectancy is 10 to 15 years. […] Treatment for PBC depends highly on what stage the disease is in.

• #56

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

• #57 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  PBC may progress to cirrhosis, this implies that there is significant scarring (fibrosis) in the liver with the formation of nodules. […] Many people who have cirrhosis will remain well with no complications or side-effects; this is termed Compensated Cirrhosis. […] The term Decompensated Cirrhosis means that the patient is developing complications of the cirrhosis. […] Patients with cirrhosis, of whatever cause, are at higher risk of developing cancer of the liver (hepatocellular carcinoma but also known as hepatoma). […] When the liver fails to clear the toxins from the bowel, the toxins will enter the circulation and may cause hepatic encephalopathy (HE). […] Ascites is the build-up of fluid in the abdomen. […] There are many causes of ankle swelling (pedal oedema). […] Varices are dilated veins which can develop as a consequence of advanced scarring and cirrhosis, whatever the cause of the cirrhosis. […] Jaundice is the result of a build-up of bilirubin in the blood and leads to a yellow colour in the eyes, and skin. […] Portal hypertension can be a consequence of cirrhosis.

• #58

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

• #59

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

  But as the liver slowly becomes more scarred and damaged (eventually leading to cirrhosis), you may have additional symptoms. […] These can include: yellowing of the whites of the eyes and the skin (jaundice), although this may be less noticeable on brown or black skin, a build-up of fluid in the legs, ankles and feet (oedema), build-up of fluid in your tummy that can make you look heavily pregnant (ascites), the formation of small fatty deposits on the skin, usually around your eyes (xanthelasmata), dark urine and pale stools, a tendency to bleed and bruise more easily, problems with memory and concentration.

• #60 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  As liver damage worsens, primary biliary cholangitis can cause serious health problems, including: Liver scarring, called cirrhosis. Cirrhosis makes it difficult for your liver to work and may lead to liver failure. It means the later stage of primary biliary cholangitis. People with primary biliary cholangitis and cirrhosis have a poor medical outlook. They also have a higher risk of other complications.

• #61 Disease Progression of PBC

  https://www.hcplive.com/view/disease-progression-of-pbc

  I describe this as a chronic inflammatory liver disease state. While we all have taken a test question as physicians that this attacks the medium-sized bile ducts, and they have a positive autoantibody, what you have to understand is we look at is as a chronic inflammatory state that, over time, in many patients can lead to chronic and permanent liver damage or cirrhosis. The trajectory there is defined by the activity of the disease, as we mentioned, whether its a highly active patient, meaning theyre difficult to control from their laboratory parameters, or the inflammation of the liver. From there, how long it is, is based on our modeling data that I think well talk about later. […] With the end-stage liver disease, they present I think a bit worse than other disease states. They have the extrahepatic manifestations of PBC, on top of the decompensation with ascites, varices, and portal hypertension that all cirrhotics deal with. They have the additional problems of the chronic extrahepatic manifestations of this disease. When you start seeing signs of portal hypertension, increased problems with low platelets, ascites, and those types of things, it is a sign that the disease has progressed to a permanent state and does need to be evaluated for a possible transplant.

• #62 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  PBC may progress to cirrhosis, this implies that there is significant scarring (fibrosis) in the liver with the formation of nodules. […] Many people who have cirrhosis will remain well with no complications or side-effects; this is termed Compensated Cirrhosis. […] The term Decompensated Cirrhosis means that the patient is developing complications of the cirrhosis. […] Patients with cirrhosis, of whatever cause, are at higher risk of developing cancer of the liver (hepatocellular carcinoma but also known as hepatoma). […] When the liver fails to clear the toxins from the bowel, the toxins will enter the circulation and may cause hepatic encephalopathy (HE). […] Ascites is the build-up of fluid in the abdomen. […] There are many causes of ankle swelling (pedal oedema). […] Varices are dilated veins which can develop as a consequence of advanced scarring and cirrhosis, whatever the cause of the cirrhosis. […] Jaundice is the result of a build-up of bilirubin in the blood and leads to a yellow colour in the eyes, and skin. […] Portal hypertension can be a consequence of cirrhosis.

• #63 Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

  https://www.gutnliver.org/journal/view.html?pn=ahead&uid=2179&vmd=Full

  Primary biliary cholangitis (PBC) is a relatively common autoimmune liver disease. Histologically, PBC manifests as chronic cholestasis which is characterized by non-suppurative progressive injury to the small intrahepatic bile ducts. It is generally divided into four phases: phase I, cholangitis; phase II, peri-congestive cholangitis; phase III, progressive fibrosis; and phase IV, cirrhosis. Portal hypertension (PH) refers to is an elevated pressure in the portal vein due to various causes, and it is associated with clinical manifestations such as splenomegaly, hypersplenism, esophagogastric fundal varices and ascites, etc. As PBC progresses, it can eventually lead to biliary cirrhosis, resulting in a variety of complications, including PH. However, there exists a subset of patients with PBC who have not yet developed cirrhosis histologically but with PH and may experience with severe complications, including splenomegaly, hypersplenism, and even gastrointestinal hemorrhage. Related studies have shown that 5% to 10% of patients with precirrhotic PBC develop esophageal varices under endoscopy. Their results suggest that low platelets (PLT), low albumin (ALB), and high total bilirubin (TBIL) are independent predictors for the development of esophageal varices in early PBC. Furthermore studies have shown that PH in early PBC is considered to be presinusoidal PH, which may be associated with nodular regenerative hyperplasia. A study of hepatic venous pressure gradient (HVPG) measurements in PBC patients shows that about 34% of precirrhotic PBC patients have high-risk PH, which is associated with portal vein and hepatic sinusoid lesions.

• #64 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  PBC may progress to cirrhosis, this implies that there is significant scarring (fibrosis) in the liver with the formation of nodules. […] Many people who have cirrhosis will remain well with no complications or side-effects; this is termed Compensated Cirrhosis. […] The term Decompensated Cirrhosis means that the patient is developing complications of the cirrhosis. […] Patients with cirrhosis, of whatever cause, are at higher risk of developing cancer of the liver (hepatocellular carcinoma but also known as hepatoma). […] When the liver fails to clear the toxins from the bowel, the toxins will enter the circulation and may cause hepatic encephalopathy (HE). […] Ascites is the build-up of fluid in the abdomen. […] There are many causes of ankle swelling (pedal oedema). […] Varices are dilated veins which can develop as a consequence of advanced scarring and cirrhosis, whatever the cause of the cirrhosis. […] Jaundice is the result of a build-up of bilirubin in the blood and leads to a yellow colour in the eyes, and skin. […] Portal hypertension can be a consequence of cirrhosis.

• #65 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. Build up of bile within the liver and blood stream causes symptoms such as nausea, jaundice and itching. […] Early stages of PBC might not show noticeable symptoms, but if picked up at a later stage, or as the disease progresses, you might experience: […] Fatigue: Feeling tired even with sufficient rest, which can affect quality of life significantly […] Itchy Skin (pruritis): Intense itching all over the body, especially at night […] Jaundice: Yellowing of the skin and eyes […] If left untreated, PBC can lead to serious complications such as: […] Cirrhosis: Severe scarring of the liver tissue, which impairs liver function […] Liver Failure: Severe impairment of liver function, which may necessitate a liver transplant. […] While there is no cure for PBC, several treatments can help manage the disease and slow its progression. […] Early diagnosis, appropriate medical management, and lifestyle changes can make a significant difference in managing the disease and maintaining liver health.

• #66 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  PBC may progress to cirrhosis, this implies that there is significant scarring (fibrosis) in the liver with the formation of nodules. […] Many people who have cirrhosis will remain well with no complications or side-effects; this is termed Compensated Cirrhosis. […] The term Decompensated Cirrhosis means that the patient is developing complications of the cirrhosis. […] Patients with cirrhosis, of whatever cause, are at higher risk of developing cancer of the liver (hepatocellular carcinoma but also known as hepatoma). […] When the liver fails to clear the toxins from the bowel, the toxins will enter the circulation and may cause hepatic encephalopathy (HE). […] Ascites is the build-up of fluid in the abdomen. […] There are many causes of ankle swelling (pedal oedema). […] Varices are dilated veins which can develop as a consequence of advanced scarring and cirrhosis, whatever the cause of the cirrhosis. […] Jaundice is the result of a build-up of bilirubin in the blood and leads to a yellow colour in the eyes, and skin. […] Portal hypertension can be a consequence of cirrhosis.

• #67 Predicting outcome in primary biliary cirrhosis | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-predicting-outcome-in-primary-biliary-S1665268119308385

  Once symptoms appear, survival of initially asymptomatic patients is comparable with survival of patients who initially presented with symptoms. […] The ability to reliably predict outcome in patients with PBC is critically important in clinical management and an essential requirement for patient counselling and timing of diagnostic procedures and therapeutic interventions. […] Male gender and advanced disease are the most frequently reported risk factors for HCC in PBC.

• #68 Living with PBC: A Patient’s Journey with a Rare Liver Disease – Society for Women’s Health Research

  https://swhr.org/living-with-pbc-a-patients-journey-with-a-rare-liver-disease/

  After diagnosis probably the first five years things were going well, she said. I took my meds, there was not a lot of noticeable symptoms, and I was feeling fine. […] As the years continued, however, Garza began to experience flare-ups in her condition, often related to stress or changes in routine. […] But then, in 2018, Garza found herself in the hospital with a variceal bleed, a possible indication of cirrhosis. […] Its progressive, and over time I may need a transplant.

• #69 Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

  https://www.gutnliver.org/journal/view.html?pn=ahead&uid=2179&vmd=Full

  Primary biliary cholangitis (PBC) is a relatively common autoimmune liver disease. Histologically, PBC manifests as chronic cholestasis which is characterized by non-suppurative progressive injury to the small intrahepatic bile ducts. It is generally divided into four phases: phase I, cholangitis; phase II, peri-congestive cholangitis; phase III, progressive fibrosis; and phase IV, cirrhosis. Portal hypertension (PH) refers to is an elevated pressure in the portal vein due to various causes, and it is associated with clinical manifestations such as splenomegaly, hypersplenism, esophagogastric fundal varices and ascites, etc. As PBC progresses, it can eventually lead to biliary cirrhosis, resulting in a variety of complications, including PH. However, there exists a subset of patients with PBC who have not yet developed cirrhosis histologically but with PH and may experience with severe complications, including splenomegaly, hypersplenism, and even gastrointestinal hemorrhage. Related studies have shown that 5% to 10% of patients with precirrhotic PBC develop esophageal varices under endoscopy. Their results suggest that low platelets (PLT), low albumin (ALB), and high total bilirubin (TBIL) are independent predictors for the development of esophageal varices in early PBC. Furthermore studies have shown that PH in early PBC is considered to be presinusoidal PH, which may be associated with nodular regenerative hyperplasia. A study of hepatic venous pressure gradient (HVPG) measurements in PBC patients shows that about 34% of precirrhotic PBC patients have high-risk PH, which is associated with portal vein and hepatic sinusoid lesions.

• #70 Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

  https://www.gutnliver.org/journal/view.html?pn=ahead&uid=2179&vmd=Full

  Approximately 24.2% of precirrhotic PBC patients have PH, which is histologically related to the injury of bile ducts. High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values are associated with increased risk of precirrhotic PBC with PH. Compared to the non-PH group, patients in the PH group showed a higher prevalence of obliterative portal venopathy, incomplete septal fibrosis, portal tract abnormalities and non-zonal sinusoidal dilatation. In terms of clinical manifestations, patients with PH were more likely to show jaundice, ascites, and epigastric discomfort. Due to the reduced anabolic function of the liver in combined PH, ALB synthesis is reduced, plasma colloid osmotic pressure is reduced, and portal vein pressure is increased, which leads to the formation of ascites. When the intrahepatic bile duct is injured, the ability to convert indirect bilirubin into direct bilirubin decreases, resulting in the abnormal excretion of bilirubin, which leads to jaundice. The study showed that jaundice indicates a considerable degree of destruction of the intrahepatic bile duct, which may cause a strong inflammatory response to hepatic regenerative nodules and portal vein, leading to PH.

• #71 Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

  https://www.gutnliver.org/journal/view.html?pn=ahead&uid=2179&vmd=Full

  Approximately 24.2% of precirrhotic PBC patients have PH, which is histologically related to the injury of bile ducts. High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values are associated with increased risk of precirrhotic PBC with PH. Compared to the non-PH group, patients in the PH group showed a higher prevalence of obliterative portal venopathy, incomplete septal fibrosis, portal tract abnormalities and non-zonal sinusoidal dilatation. In terms of clinical manifestations, patients with PH were more likely to show jaundice, ascites, and epigastric discomfort. Due to the reduced anabolic function of the liver in combined PH, ALB synthesis is reduced, plasma colloid osmotic pressure is reduced, and portal vein pressure is increased, which leads to the formation of ascites. When the intrahepatic bile duct is injured, the ability to convert indirect bilirubin into direct bilirubin decreases, resulting in the abnormal excretion of bilirubin, which leads to jaundice. The study showed that jaundice indicates a considerable degree of destruction of the intrahepatic bile duct, which may cause a strong inflammatory response to hepatic regenerative nodules and portal vein, leading to PH.

• #72 Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

  https://www.gutnliver.org/journal/view.html?pn=ahead&uid=2179&vmd=Full

  Approximately 24.2% of precirrhotic PBC patients have PH, which is histologically related to the injury of bile ducts. High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values are associated with increased risk of precirrhotic PBC with PH. Compared to the non-PH group, patients in the PH group showed a higher prevalence of obliterative portal venopathy, incomplete septal fibrosis, portal tract abnormalities and non-zonal sinusoidal dilatation. In terms of clinical manifestations, patients with PH were more likely to show jaundice, ascites, and epigastric discomfort. Due to the reduced anabolic function of the liver in combined PH, ALB synthesis is reduced, plasma colloid osmotic pressure is reduced, and portal vein pressure is increased, which leads to the formation of ascites. When the intrahepatic bile duct is injured, the ability to convert indirect bilirubin into direct bilirubin decreases, resulting in the abnormal excretion of bilirubin, which leads to jaundice. The study showed that jaundice indicates a considerable degree of destruction of the intrahepatic bile duct, which may cause a strong inflammatory response to hepatic regenerative nodules and portal vein, leading to PH.

• #73 Primary Biliary Cholangitis (PBC) – Liver and Gallbladder Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/liver-and-gallbladder-disorders/fibrosis-and-cirrhosis-of-the-liver/primary-biliary-cholangitis-pbc

  Eventually, any of the symptoms and complications of cirrhosis can develop. […] Primary biliary cholangitis usually progresses slowly, although how fast it progresses varies greatly. Symptoms may not appear for 2 years or for up to 10 to 15 years. Some people become very ill in 3 to 5 years. Once symptoms develop, life expectancy is about 10 years. […] Certain characteristics suggest that the disorder will progress quickly: Rapidly worsening symptoms, older age, accumulation of fluid and other symptoms of cirrhosis, presence of an autoimmune disorder, such as rheumatoid arthritis, certain abnormal liver test results. […] Once jaundice develops, death may occur within months.

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

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

  PBC is chronic and progressive. Its not curable, and over time it can lead to permanent liver damage. […] PBC often develops slowly, however. This means you may be able to remain healthy for many years with few symptoms. Once you develop symptoms, medication may help manage them. […] 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.

• #75 Predicting outcome in primary biliary cirrhosis | Annals of Hepatology

  https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-predicting-outcome-in-primary-biliary-S1665268119308385

  Once symptoms appear, survival of initially asymptomatic patients is comparable with survival of patients who initially presented with symptoms. […] The ability to reliably predict outcome in patients with PBC is critically important in clinical management and an essential requirement for patient counselling and timing of diagnostic procedures and therapeutic interventions. […] Male gender and advanced disease are the most frequently reported risk factors for HCC in PBC.

• #76 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Patients with primary biliary cholangitis have various clinical findings on physical exam, which usually correlate with the stage of disease at presentation. About 40% of patients have skin complaints such as dry skin, hyperpigmentation, xanthelasma, xanthomas, jaundice, dermatographism, and fungal infection of the feet. […] The goal of therapy in primary biliary cholangitis is to prevent disease progression and manage the symptoms and complications related to chronic cholestasis. […] The most reliable indicators of a patient’s prognosis from primary biliary cholangitis are the rise in serum bilirubin level and the Mayo risk score. […] Almost all patients with PBC develop moderate-to-severe fatigue, which persists even after a liver transplant. Universally, once patients develop symptoms, the outcome is grim.

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

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

  Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disorder characterized by progressive cholestatic that, if untreated, can progress to liver fibrosis, cirrhosis and liver decompensation requiring liver transplant. […] PBC is a progressive hepatic disease and the early stages of the disease manifest with fatigue, pruritus, right upper quadrant abdominal discomfort and jaundice. […] The introduction of ursodeoxycholic acid (UDCA) in the treatment of PBC patients has changed the disease course, improving transplant-free survival rates to up to 60% of patients, thereby reducing mortality. Currently, life expectancy in PBC patients under UDCA therapy is similar to non-PBC patients. […] PBC is considered an organ-specific autoimmune disorder and, along with the autoimmune hepatitis (AIH) and the primary sclerosing cholangitis (PSC), is classified as an autoimmune liver disease.

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

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

  Primary biliary cirrhosis (PBC) is a cholestatic liver disease in which 90% of patients are women, usually between age 30 and 65 at the time of diagnosis. […] Fatigue is the most common symptom reported by patients with PBC and affects up to 78% of patients. Fatigue does not correlate with the severity of disease nor the histologic stage of liver injury on biopsy. […] The second most common symptom in PBC occurring in 20-70% of patients is pruritus (itching) which may be intermittent and worse at night, often exacerbated with contact to fabrics, heat and pregnancy. […] Late in the course of PBC, portal hypertension with variceal hemorrhage/portal gastropathy can occur sometimes before there is cirrhosis. […] The features of advanced PBC are no different from those of other causes of cirrhosis and include muscle wasting, weakness, gastrointestinal bleeding, ascites, encephalopathy, the hepatopulmonary syndrome, and hepatocellular carcinoma. […] Without therapy, the 10 year survival of asymptomatic patients ranges from 50%-70%, while the median survival for symptomatic patients ranges from 5-8 years.

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

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

  Many people with PBC have no symptoms in the early stages. As the disease progresses, signs of biliary disease begin to appear. The earliest and most common symptoms of people with PBC are: Fatigue (65%). Itchy skin (55%). […] However, not everyone discovers PBC in time to treat it in the early stages. And some people simply have a more aggressive form of the disease. Higher levels of fatigue and higher levels of bilirubin in your blood predict a faster progression to liver failure. If you reach this stage, you’ll need a liver transplant to survive. But for those with PBC who have successful liver transplants, the prognosis is excellent. […] It takes an average of 15 to 20 years for PBC to progress to the terminal stage. The first stage, when you test positive for PBC but you don’t yet have symptoms, can last a long time. About half of people will begin having symptoms in the next five to 10 years. Once you have symptoms, the average life expectancy is about 10 years. For those who have successful liver transplants, the 10-year survival rate is 65%.

• #80 Early-Stage Primary Biliary Cholangitis: Progression and Outcomes – Gastroenterology Advisor

  https://www.gastroenterologyadvisor.com/news/early-stage-primary-biliary-cholangitis-progression-and-outcomes/

  Nearly half of patients with early-stage PBC progress to a more severe stage within 5 years. […] Progression is associated with increased risk of a clinical event, so surveillance is important for patients with early-stage PBC. […] Approximately half of patients with early-stage PBC progress to a more severe stage of the disease within 5 years. […] Disease progression is associated with an increased risk of experiencing a clinical event such as liver failure, requiring liver transplantation, indicating that careful surveillance is crucial in the early stages of PBC. […] Of the 1615 patients, 904 progressed to moderate PBC and 201 patients progressed to advanced PBC over the course of the study. […] The proportion of patients transitioning to moderate-stage PBC at 1 year was 12.9%, compared with 30.2% at 3 years and 45.8% at 5 years.

• #81 Early-Stage Primary Biliary Cholangitis: Progression and Outcomes – Gastroenterology Advisor

  https://www.gastroenterologyadvisor.com/news/early-stage-primary-biliary-cholangitis-progression-and-outcomes/

  The proportion transitioning to advanced-stage PBC at 1 year was 3.4%, compared with 12.5% at 3 years and 16% at 5 years. […] The median transition time from early- to moderate-stage PBC was 2.5 years, and the median transition time from moderate- to advanced-stage PBC was 1.5 years. […] During follow-up, 236 participants experienced at least one clinical event. […] The proportions of patients with moderate-stage PBC and clinical event-free survival at 1, 3, and 5 years were 97.9%, 95.1%, and 91.5%, respectively, and the proportions of patients with advanced-stage PBC and clinical event-free survival at 1, 3, and 5 years were 90.6%, 71.2%, and 58.3%, respectively. […] An increased risk for a clinical event was found for participants transitioning from early- to moderate-stage PBC, from moderate- to advanced-stage PBC, and overall from early- to advanced-stage PBC. […] These transitions are associated with an increased probability of clinical events.

• #82 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  Symptom burden in PBC, including itch and fatigue can have a huge physical and mental impact on a patients quality of life; affecting daily activities, and even leaving some feeling isolated. […] The most common PBC symptoms are itch fatigue. […] ~60% of PBC patients are asymptomatic at time of diagnosis. […] Not everyone with PBC will experience all, or any symptoms. […] Severity of symptoms do not correlate with severity of disease. […] PBC symptoms, the most common ones being itch and fatigue, often increase and decrease in cycles. […] Whilst both pruritus and fatigue can occur at any point in the course of PBC, and do not correlate with severity of disease or with progression, there are a number of symptoms that can affect PBC patients that are associated with a more advanced liver disease.

• #83 Primary Biliary Cholangitis (PBC) – Looking Beyond the Liver | Ipsen Rare Diseases

  https://www.ipsen.com/rare-diseases/primary-biliary-cholangitis-pbc-looking-beyond-the-liver/

  Symptom burden is the main issue for the patient. […] Primary Biliary Cholangitis (PBC) is a rare, chronic, autoimmune liver disease. It can significantly impact a persons quality of life and, if untreated, can result in liver failure or premature death. […] Those living with PBC face symptoms such as itch (pruritis), fatigue, and jaundice, and are significantly more likely to face limitations in their daily life, such as pursuing hobbies and playing sports. Itch and fatigue have the most significant negative impact on quality of life, impacting 70% and 95% of patients respectively. Severe itch can lead to sleep deprivation, depression, and suicidal ideation in some cases. […] Patients tell us they are concerned others will think they have an infectious disease that is contagious, so theyre scared and afraid of being out in public, and it affects their lives with their partners at home, and with their jobs outside the house. […] We not only manage the liver disease, but also all these extrahepatic manifestations we see in our patients. And to really look at this as two different issues, we have to optimize, to address, to treat, as far as possible.

• #84 Primary Biliary Cholangitis (PBC) – Looking Beyond the Liver | Ipsen Rare Diseases

  https://www.ipsen.com/rare-diseases/primary-biliary-cholangitis-pbc-looking-beyond-the-liver/

  Symptom burden is the main issue for the patient. […] Primary Biliary Cholangitis (PBC) is a rare, chronic, autoimmune liver disease. It can significantly impact a persons quality of life and, if untreated, can result in liver failure or premature death. […] Those living with PBC face symptoms such as itch (pruritis), fatigue, and jaundice, and are significantly more likely to face limitations in their daily life, such as pursuing hobbies and playing sports. Itch and fatigue have the most significant negative impact on quality of life, impacting 70% and 95% of patients respectively. Severe itch can lead to sleep deprivation, depression, and suicidal ideation in some cases. […] Patients tell us they are concerned others will think they have an infectious disease that is contagious, so theyre scared and afraid of being out in public, and it affects their lives with their partners at home, and with their jobs outside the house. […] We not only manage the liver disease, but also all these extrahepatic manifestations we see in our patients. And to really look at this as two different issues, we have to optimize, to address, to treat, as far as possible.

• #85

  https://journals.lww.com/hep/fulltext/9900/primary_biliary_cholangitis__personalizing.1086.aspx

  While using pharmacotherapy to modify the progression of PBC, clinicians should continuously address symptoms: fatigue and pruritus, especially, can be very pervasive and significantly impair quality of life. […] The most important predictor of poor PBC-related quality of life is social dysfunction, which is directly related to the presence of symptoms. […] Younger patients are at increased risk and consistently report poorer global PBC-related quality of life. This association may be explained by insufficient coping skills and a lack of support networks in that age group. […] A key development in the past decade has been the understanding that a subgroup of UDCA-treated patients are at higher risk of disease progression, and the recognition that serum alkaline phosphatase (ALP) levels while on UDCA treatment predict risk of liver transplant or death independent of serum bilirubin levels.

• #86 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.international/what-is-pbc/symptoms/

  Symptom burden in PBC, including itch and fatigue can have a huge physical and mental impact on a patients quality of life; affecting daily activities, and even leaving some feeling isolated. […] The most common PBC symptoms are itch fatigue. […] ~60% of PBC patients are asymptomatic at time of diagnosis. […] Not everyone with PBC will experience all, or any symptoms. […] Severity of symptoms do not correlate with severity of disease. […] PBC symptoms, the most common ones being itch and fatigue, often increase and decrease in cycles. […] Whilst both pruritus and fatigue can occur at any point in the course of PBC, and do not correlate with severity of disease or with progression, there are a number of symptoms that can affect PBC patients that are associated with a more advanced liver disease.

• #87 Symptoms of PBC. PBC Foundation.

  https://www.pbcfoundation.org.uk/what-is-pbc/symptoms/

  Symptom burden in PBC, including itch and fatigue can have a huge physical and mental impact on a patients quality of life; affecting daily activities, and even leaving some feeling isolated. […] The most common PBC symptoms are itch fatigue. […] ~60% of PBC patients are asymptomatic at time of diagnosis. […] Not everyone with PBC will experience all, or any symptoms. […] Severity of symptoms do not correlate with severity of disease. […] Patients can take an active role in the management of their symptoms. […] Treatments self-care practices have been shown to improve symptoms. […] PBC symptoms, the most common ones being itch and fatigue, often increase and decrease in cycles. […] Whilst both pruritus and fatigue can occur at any point in the course of PBC, and do not correlate with severity of disease or with progression, there are a number of symptoms that can affect PBC patients that are associated with a more advanced liver disease.

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

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

  Your healthcare professional will ask you about your health history and your family’s health history, and perform a physical exam. The following tests and procedures may be used to diagnose primary biliary cholangitis. […] Blood tests may be done to check for anti-mitochondrial antibodies, also known as AMAs. These substances almost never occur in people without the disease, even if they have other liver disorders. Therefore, a positive AMA test is considered a very reliable sign of the disease. However, a small number of people with primary biliary cirrhosis don’t have AMAs. […] More than half the people with primary biliary cholangitis have extreme increases in blood fats, including total cholesterol level. […] There’s no cure for primary biliary cholangitis, but medicines are available to help slow the progression of the disease and prevent complications.

• #89 Primary Biliary Cholangitis – Liver Foundation

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

  Primary Biliary Cholangitis (PBC) is a rare condition that affects the small bile ducts (the network of small tubes that carry bile in the liver) […] Over time, the bile ducts can become inflamed and scarred […] PBC may eventually lead to long-term liver damage and liver failure […] Most people are not aware they have PBC and do not have symptoms […] If you do have symptoms, they usually start with feeling very tired (fatigue) and having itchy skin […] As the PBC gets worse, other symptoms may start […] PBC is a chronic disease (it lasts for a long time) and without treatment it gradually gets worse […] Over 15 to 20 years, someone with PBC can slowly develop liver failure […] Most people with PBC have a very good outcome if they have ongoing regular treatment […] Some patients, however, do not respond to treatment and will develop complications

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

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

  When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. […] Primary biliary cholangitis causes fatigue. […] Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable. […] If you have high cholesterol levels in your blood, your healthcare team may recommend taking a medicine known as a statin to help lower your levels. […] Certain complications are commonly associated with primary biliary cholangitis.

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

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

  Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disorder characterized by progressive cholestatic that, if untreated, can progress to liver fibrosis, cirrhosis and liver decompensation requiring liver transplant. […] PBC is a progressive hepatic disease and the early stages of the disease manifest with fatigue, pruritus, right upper quadrant abdominal discomfort and jaundice. […] The introduction of ursodeoxycholic acid (UDCA) in the treatment of PBC patients has changed the disease course, improving transplant-free survival rates to up to 60% of patients, thereby reducing mortality. Currently, life expectancy in PBC patients under UDCA therapy is similar to non-PBC patients. […] PBC is considered an organ-specific autoimmune disorder and, along with the autoimmune hepatitis (AIH) and the primary sclerosing cholangitis (PSC), is classified as an autoimmune liver disease.

• #92 Primary biliary cholangitis – Wikipedia

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

  PBC can eventually progress to cirrhosis of the liver. This, in turn, may lead to a number of symptoms or complications, including fluid retention in the abdomen (ascites) in more advanced disease, enlarged spleen in more advanced disease, oesophageal varices in more advanced disease, and hepatic encephalopathy, including coma in extreme cases in more advanced disease. […] The introduction of UDCA has dramatically changed the pattern and the course of the disease. […] Among the UDCA-treated patients, the degree of the liver biochemistry improvement, i.e. the UDCA-response, identifies patients with different long-term prognosis. […] Survival is significantly reduced though, in those with abnormal liver biochemistry on treatment. […] Hepatocellular carcinoma (HCC) is infrequent in PBC. Recent large-scale cohort studies highlighted that the lack of UDCA-response after 12 months of therapy and male sex are associated with increased future risk of developing HCC in PBC.

• #93

  https://journals.lww.com/hep/fulltext/9900/primary_biliary_cholangitis__personalizing.1086.aspx

  Practice guidance documents endorsed by the American Association for the Study of Liver Diseases and European Association for Study of the Liver recommend assessing for response to UDCA treatment after 12 months of therapy, at which point a second-line therapy should be considered. […] Despite the established efficacy and safety of UDCA as a first-line treatment for PBC, up to 40% of patients do not achieve an adequate biochemical response, and even fewer achieve normalization of their ALP. […] PPARs consist of 3 primary isoforms, PPAR, PPAR, and PPAR, which differ in tissue expression, ligand binding, and genes that are induced, but regardless of PPAR specificities, multiple PPAR agonists have demonstrated anticholestatic effects in patients with PBC. […] A particular advantage of PPAR agonists over FXR agonists is their ability to improve rather than exacerbate cholestatic pruritus. […] At present, the lack of comparative effectiveness data precludes ranking these options, and choice will be based on the individual patient profile-disease stage, presence of symptoms and comorbidities, potential drug-drug interactions, patient preference, and drug availability and affordability.

• #94

  https://journals.lww.com/hep/fulltext/9900/primary_biliary_cholangitis__personalizing.1086.aspx

  Primary biliary cholangitis (PBC) is an enigmatic, autoimmune disease targeting the small intralobular bile ducts resulting in cholestasis and potentially progression to biliary cirrhosis. […] Clinically, the disease manifests primarily by fatigue and pruritus. While most patients are asymptomatic at the time of diagnosis, these symptoms will afflict up to 70% of patients at some point in their lives. Fatigue, the most disabling symptom in PBC, is often associated with cognitive impairment, in addition to social and emotional dysfunction. […] Moreover, sicca symptoms are present in up to a third of patients. […] Unfortunately, however, UDCA does not help control the symptoms of PBC, which remains an enormous unmet need. Furthermore, a sizeable proportion of patients fail to demonstrate a robust biochemical response to UDCA or are intolerant to UDCA and continue to progress toward cirrhosis and its complications.

• #95

  https://journals.lww.com/hep/fulltext/9900/primary_biliary_cholangitis__personalizing.1086.aspx

  Practice guidance documents endorsed by the American Association for the Study of Liver Diseases and European Association for Study of the Liver recommend assessing for response to UDCA treatment after 12 months of therapy, at which point a second-line therapy should be considered. […] Despite the established efficacy and safety of UDCA as a first-line treatment for PBC, up to 40% of patients do not achieve an adequate biochemical response, and even fewer achieve normalization of their ALP. […] PPARs consist of 3 primary isoforms, PPAR, PPAR, and PPAR, which differ in tissue expression, ligand binding, and genes that are induced, but regardless of PPAR specificities, multiple PPAR agonists have demonstrated anticholestatic effects in patients with PBC. […] A particular advantage of PPAR agonists over FXR agonists is their ability to improve rather than exacerbate cholestatic pruritus. […] At present, the lack of comparative effectiveness data precludes ranking these options, and choice will be based on the individual patient profile-disease stage, presence of symptoms and comorbidities, potential drug-drug interactions, patient preference, and drug availability and affordability.

• #96

  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 does not always cause symptoms, but some people may experience: bone and joint aches, fatigue (extreme tiredness), itchy skin, dry eyes and mouth, pain or discomfort in the upper right side of their tummy. […] PBC is a progressive condition, which means the damage to the liver can steadily get worse over time. […] The rate at which PBC progresses varies between individuals. Sometimes, it can take decades. […] 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.

• #97 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.

• #98 Symptoms & Causes of Primary Biliary Cholangitis (Primary Biliary Cirrhosis) – NIDDK

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

  The most common symptoms of primary biliary cholangitis are feeling tired and having itchy skin. […] As the disease gets worse, symptoms may include darkening of skin color, fatty deposits that appear as yellow bumps on the skin, called xanthomas, and symptoms of cirrhosis, such as edema, jaundice, and weight loss. […] Many people have no symptoms when they are first diagnosed with primary biliary cholangitis. Doctors diagnose about 60% of people with primary biliary cholangitis before symptoms begin. People with primary biliary cholangitis and no symptoms are identified through blood tests. Some people do not have symptoms for years after they have been diagnosed with primary biliary cholangitis.

• #99 Primary biliary cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis/symptoms-causes/syc-20376874

  Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis. […] More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes. […] Common early symptoms include: Fatigue. Itchy skin. […] Later signs and symptoms may include: Yellowing of the skin and eyes, called jaundice. Dry eyes and mouth. Pain in the upper right abdomen. Swelling of the spleen, called splenomegaly. Bone, muscle or joint pain. Swollen feet and ankles. Buildup of fluid in the abdomen due to liver failure, called ascites. Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees. Darkening of the skin that’s not related to sun exposure, called hyperpigmentation. Weak and brittle bones, called osteoporosis, which can lead to fractures. High cholesterol. Diarrhea that may include greasy stools, called steatorrhea. Underactive thyroid, called hypothyroidism. Weight loss.

• #100 Primary Biliary Cholangitis: Symptoms & Causes

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

  Thinning of the bones (osteoporosis) leading to fractures is another complication of Primary Biliary Cholangitis (PBC). […] While this is more common in late stages of the disease, it can occur earlier as well. […] Keep in mind that Primary Biliary Cholangitis (PBC) usually advances slowly over a period of years. Many people lead normal lives for years without symptoms, depending on how early the diagnosis is made. And while there is no cure, people are having good results slowing disease progression and living longer without complications by adhering to their medication regimen and maintaining a healthy lifestyle.

• #101 Primary Biliary Cholangitis (PBC)

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

  Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic inflammatory autoimmune liver disease, affecting the small intra-hepatic bile ducts. Over time, PBC can lead to fibrosis and eventually to cirrhosis. […] Many patients are asymptomatic at the time of diagnosis. Fatigue and itching are frequently reported throughout the course of the disease. Additional symptoms can include difficulty in concentration, joint pain, and pain in the upper right quadrant of the abdomen. […] An early diagnosis is essential to enable treatment to prevent or slow down the progression of the disease. Patients who are diagnosed early and who show a good response to treatment have a life expectancy comparable to individuals without the disease. […] The standard first line treatment for PBC is ursodeoxycholic acid (UDCA), which is usually very well tolerated.

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