Materiały źródłowe

• #1 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. […] It’s not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors. […] The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver. […] Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

• #2 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. […] Researchers theorize that patient with primary biliary cirrhosis has both genetic predispositions and the right environmental trigger. The prevalence of the disease is 100-fold higher in first-degree relatives of the index patient, which strongly suggests a genetic predisposition. […] The inflammation is thought to result from a direct insult of environmental factors and toxins. […] The pathogenesis of primary biliary cholangitis is thought to be related to the interaction between genetic predisposition and environmental triggers. […] The genetic predisposition is suggested by a strong prevalence of the disease in first-degree relatives, with an odds ratio of 11.

• #3 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. […] It’s not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors. […] The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver. […] Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

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

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

  Primary biliary cholangitis is a chronic and progressive condition that causes inflammation and, eventually, the destruction of the bile ducts that run through your liver. […] Primary biliary cholangitis (PBC) is a liver disease that affects the bile ducts that run through your liver. […] PBC was formerly known as primary biliary cirrhosis. […] Cholangitis means inflammation in your bile ducts. […] This means that the disease itself is the original cause of inflammation in your bile ducts. […] In PBC, it appears that your own immune system attacks the cells lining your intrahepatic bile ducts, causing inflammation. […] This is called autoimmune disease. […] We dont know why autoimmune disease occurs. […] There does appear to be a genetic factor. […] There also seems to be an environmental factor, meaning that it may take something in your environment to trigger the disease.

• #5 Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-diagnosis-and-prognosis-of-primary-biliary-cholangitis

  Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis and eventually may result in cirrhosis and liver failure. […] The terminology was changed from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disorder and its natural history. […] The pathogenesis of PBC, the treatment of PBC, and the treatment of pruritus due to cholestasis are discussed separately. […] Primary biliary cholangitis (PBC) is rare, with a reported prevalence of 19 to 402 cases per million persons. […] The vast majority of patients (90 to 95 percent) are women, and most patients are diagnosed between the ages of 30 and 65 years.

• #6 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. […] It’s not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors. […] The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver. […] Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

• #7 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Several human leukocyte antigen (HLA) allele associations have been reported with primary biliary cirrhosis, which includes DRB1, DR3, DPB1, DQA1, and DQB1. […] The environmental triggers include toxic waste, cigarette smoking, nail polish, hair dye, and various xenobiotics. […] These environmental triggers induce the autoimmune reaction in genetically susceptible patients, which is evident by the presence of a humoral and cellular response to an intracytoplasmic antigen, the presence of anti-mitochondrial antibody (highly specific), and the involvement of T lymphocytes in the destruction of bile ducts. […] Primary biliary cirrhosis is associated with highly specific autoantibodies. The anti-mitochondrial antibody is found in 85% of the cases.

• #8 Primary biliary cholangitis – Wikipedia

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

  Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. […] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. […] PBC has an immunological basis, and is classified as an autoimmune disorder. […] It results from a slow, progressive destruction of the small bile ducts of the liver, with the intralobular ducts and the canals of Hering (intrahepatic ductules) being affected early in the disease. […] Most people with PBC (more than 90%) have antimitochondrial antibodies (AMAs) against pyruvate dehydrogenase complex (PDC-E2), an enzyme complex found in the mitochondria. […] People with PBC may also have been diagnosed with another autoimmune disease, such as a rheumatological, endocrinological, gastrointestinal, pulmonary, or dermatological condition, suggesting shared genetic and immune abnormalities.

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

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

  Primary biliary cholangitis (PBC; formerly known as primary biliary cirrhosis) is an autoimmune liver disorder characterized by the progressive destruction of intrahepatic bile ducts, leading to cholestasis, cirrhosis, and liver failure. […] Primary biliary cholangitis (PBC) is the most common liver disease associated with chronic cholestasis in adults. Most (95%) cases occur in women aged 35 to 70. PBC also clusters in families. A genetic predisposition, perhaps involving the X chromosome, probably contributes. There may be an inherited abnormality of immune regulation. […] An autoimmune mechanism has been implicated; antibodies to antigens located on the inner mitochondrial membranes occur in 95% of cases. These antimitochondrial antibody (AMA), the serologic hallmarks of PBC, are not cytotoxic and are not involved in bile duct damage.

• #10 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

  PBC is associated with other autoimmune disorders, such as systemic sclerosis, Sjgren syndrome, CREST syndrome (also known as limited scleroderma), and autoimmune thyroiditis. […] T cells attack the small bile ducts. CD4 and CD8 T lymphocytes directly target biliary epithelial cells. The trigger for the immunologic attack on bile ducts is unknown. Exposure to foreign antigens, such as an infectious (bacterial or viral) or toxic agent, may be the instigating event. These foreign antigens might be structurally similar to endogenous proteins (molecular mimicry); then the subsequent immunologic reaction would be autoimmune and self-perpetuating. Destruction and loss of bile ducts lead to impaired bile formation and secretion (cholestasis). […] Chronic cholestasis thus leads to liver cell inflammation and scarring in the periportal areas. Eventually, hepatic inflammation decreases as hepatic fibrosis progresses to cirrhosis.

• #11 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. […] Researchers theorize that patient with primary biliary cirrhosis has both genetic predispositions and the right environmental trigger. The prevalence of the disease is 100-fold higher in first-degree relatives of the index patient, which strongly suggests a genetic predisposition. […] The inflammation is thought to result from a direct insult of environmental factors and toxins. […] The pathogenesis of primary biliary cholangitis is thought to be related to the interaction between genetic predisposition and environmental triggers. […] The genetic predisposition is suggested by a strong prevalence of the disease in first-degree relatives, with an odds ratio of 11.

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

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

  The causes of primary biliary cholangitis (PBC) remain unknown. But current evidence suggests that PBC occurs in people with a genetic predispositionin other words, there may be a hereditary factor. However, there may also be other factors that combine with genetics to trigger the disease. […] Medical scientists believe that PBC is an autoimmune disease. This means that your immune system, which protects against infections and cancer, mistakes cells in the bile ducts as being abnormal and attacks them. […] Some factorssuch as an infection or some form of toxic exposure from the environmentmay trigger your immune system into making this mistake. […] Its important to note that PBC is not caused by alcohol consumption. […] PBC used to be known as primary biliary cirrhosis.

• #13 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. […] Researchers theorize that patient with primary biliary cirrhosis has both genetic predispositions and the right environmental trigger. The prevalence of the disease is 100-fold higher in first-degree relatives of the index patient, which strongly suggests a genetic predisposition. […] The inflammation is thought to result from a direct insult of environmental factors and toxins. […] The pathogenesis of primary biliary cholangitis is thought to be related to the interaction between genetic predisposition and environmental triggers. […] The genetic predisposition is suggested by a strong prevalence of the disease in first-degree relatives, with an odds ratio of 11.

• #14 Primary Biliary Cirrhosis: Symptoms and Treatment | Doctor

  https://patient.info/doctor/primary-biliary-cholangitis-pro

  Primary biliary cholangitis (PBC) is a slowly progressive autoimmune disease of the biliary system with a chronic course which may extend over many decades. […] The aetiology is probably partly genetic and partly environmental: the disease is thought to be environmentally triggered in genetically predisposed individuals. […] Primary biliary cholangitis used to be called primary biliary cirrhosis, a term now confined to the late stage of PBC in which cirrhosis actually occurs. […] The triggers have yet to be determined. […] The rate of concordance amongst identical twins is amongst the highest of all autoimmune diseases. […] Families with a strong family history have been described.

• #15 Primary Biliary Cirrhosis: Symptoms and Treatment | Doctor

  https://patient.info/doctor/primary-biliary-cholangitis-pro

  Primary biliary cholangitis (PBC) is a slowly progressive autoimmune disease of the biliary system with a chronic course which may extend over many decades. […] The aetiology is probably partly genetic and partly environmental: the disease is thought to be environmentally triggered in genetically predisposed individuals. […] Primary biliary cholangitis used to be called primary biliary cirrhosis, a term now confined to the late stage of PBC in which cirrhosis actually occurs. […] The triggers have yet to be determined. […] The rate of concordance amongst identical twins is amongst the highest of all autoimmune diseases. […] Families with a strong family history have been described.

• #16 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Several human leukocyte antigen (HLA) allele associations have been reported with primary biliary cirrhosis, which includes DRB1, DR3, DPB1, DQA1, and DQB1. […] The environmental triggers include toxic waste, cigarette smoking, nail polish, hair dye, and various xenobiotics. […] These environmental triggers induce the autoimmune reaction in genetically susceptible patients, which is evident by the presence of a humoral and cellular response to an intracytoplasmic antigen, the presence of anti-mitochondrial antibody (highly specific), and the involvement of T lymphocytes in the destruction of bile ducts. […] Primary biliary cirrhosis is associated with highly specific autoantibodies. The anti-mitochondrial antibody is found in 85% of the cases.

• #17

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

  Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune disease with a progressive course that may extend over many decades. PBC is thought to be caused by a combination of genetic predisposition and environmental triggers, and is most commonly recognized in women in their 5th or 6th decade of life. […] Although there is no absolute certainty as to what causes PBC, the most widely accepted theory is that a genetically susceptible patient comes into contact with an autoimmune triggering event. This triggering event could be an environmental factor, virus, allergen, chemical or medication. There is no one trigger and every patient has their own immune triggering event. […] Genetic factors include MHC class II (DR8, DQA1*0102, DQ/1*0402), MHC class III (C4 null,c4B2), and non-MHC genes (Exon 1 of CTLA-4). Familial factors have also been identified, including PBC/positive AMA and impaired T-cell regulation, extrahepatic autoimmune diseases and a higher prevalence in people with a family member, especially an identical twin, with PBC. […] Potential environmental factors, especially in genetically vulnerable individuals, include recurrent urinary tract infections (potentially related to exposure to bacterial components or the antibiotic use), exposure to toxic chemicals and cigarette smoking.

• #18

  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. […] In PBC, the immune system (the body’s natural defence against infection and illness) mistakenly attacks the bile ducts. […] It’s not clear why this happens, but it’s thought to be caused by a combination of subtle differences in how the immune system works. […] The bile ducts become damaged and injured, causing bile to build up in the liver. This further damages the liver and may lead to scarring (cirrhosis).

• #19 What Causes Primary Biliary Cholangitis (Formerly Primary Biliary Cirrhosis)? | myPBCteam

  https://www.mypbcteam.com/resources/what-causes-primary-biliary-cholangitis-formerly-primary-biliary-cirrhosis

  Experts believe that primary biliary cholangitis (formerly known as primary biliary cirrhosis) occurs because of two main factors. First, you may be more likely to have this condition as a result of your genes. Second, PBC may develop when certain elements in your environment trigger the condition. […] Most doctors and researchers believe that PBC is an autoimmune disease, meaning the immune system attacks healthy tissues. If you have PBC, you probably have large numbers of white blood cells called T cells in your liver. T cells usually help fight infections, but in PBC, they mistakenly damage the cells that make up your bile ducts. This eventually leads to ongoing inflammation, cirrhosis (scarring of liver tissue), and possibly liver failure. […] In PBC, immune system cells mistakenly attack the bile ducts, causing inflammation, cirrhosis (scarring of liver tissue), and possibly liver failure.

• #20 Primary Biliary Cholangitis – Liver Foundation

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

  PBC is a rare medical condition. PBC stands for primary biliary cholangitis. It’s a medical condition that affects the small bile ducts in the liver. […] Over a period of time, the liver disease impacts on the bile ducts by causing inflammation and scarring called fibrosis and eventually, if untreated, this may lead to more severe fibrosis or scarring and cirrhosis of the liver. […] PBC is an autoimmune liver condition whereby the body’s own healthy immune system becomes overactive and attacks healthy liver cells and liver tissue. We do not understand why the immune system is fully activated to attack the liver in this manner. It is thought that we inherit genes that predispose us to this medical condition. […] These genes may be activated by environmental triggers such as urinary tract infections, smoking and even certain chemical exposures, although that is not fully understood.

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

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

  Infection with organisms of the Enterobacteriaceae family: Cross-reactivity between antigens on the bacterial wall and the mitochondria has been postulated. Patients with PBC present with an increased incidence of gram-negative urinary tract infections. […] Environmental substances: Certain chemicals found in such items as lipstick and hair dye can trigger the development of immunogenic antigens and the breakdown of self-antigen tolerance.

• #22 Primary biliary cholangitis (PBC) Causes, Symptoms, Diagnosis and Treatment – Cura4U

  https://cura4u.com/conditions/primary-biliary-cholangitis

  Primary biliary cholangitis (PBC) is an autoimmune disease that affects the liver. The old name of this disease is primary biliary cirrhosis. The precise cause of the disease is unknown. It tends to run in families and is thought to result from abnormal autoimmune reactions. Some environmental factors may play a role in triggering the disease, such as; smoking, infections, and chemical exposure. […] People with PBC have been found to have frequent urinary tract infections, particularly with Enterobacteriaceae bacteria, exhibiting the cross-reactivity between the bacterial parts and the body’s antigens.

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

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

  Infection with organisms of the Enterobacteriaceae family: Cross-reactivity between antigens on the bacterial wall and the mitochondria has been postulated. Patients with PBC present with an increased incidence of gram-negative urinary tract infections. […] Environmental substances: Certain chemicals found in such items as lipstick and hair dye can trigger the development of immunogenic antigens and the breakdown of self-antigen tolerance.

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

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

  Infection with organisms of the Enterobacteriaceae family: Cross-reactivity between antigens on the bacterial wall and the mitochondria has been postulated. Patients with PBC present with an increased incidence of gram-negative urinary tract infections. […] Environmental substances: Certain chemicals found in such items as lipstick and hair dye can trigger the development of immunogenic antigens and the breakdown of self-antigen tolerance.

• #25 What Causes Primary Biliary Cholangitis (Formerly Primary Biliary Cirrhosis)? | myPBCteam

  https://www.mypbcteam.com/resources/what-causes-primary-biliary-cholangitis-formerly-primary-biliary-cirrhosis

  People who are exposed to cigarette smoke are more likely to develop PBC. Smoking may also speed up the progression of PBC, possibly because of toxic chemicals in cigarettes. […] You may be more likely to be diagnosed with PBC if you regularly come into contact with toxic chemicals. For example, your risk may increase if you work with chemicals or live near a site where toxic waste is dumped. Additionally, some studies have found links between the risk of PBC and frequent use of nail polish.

• #26 What Causes Primary Biliary Cholangitis (Formerly Primary Biliary Cirrhosis)? | myPBCteam

  https://www.mypbcteam.com/resources/what-causes-primary-biliary-cholangitis-formerly-primary-biliary-cirrhosis

  People who are exposed to cigarette smoke are more likely to develop PBC. Smoking may also speed up the progression of PBC, possibly because of toxic chemicals in cigarettes. […] You may be more likely to be diagnosed with PBC if you regularly come into contact with toxic chemicals. For example, your risk may increase if you work with chemicals or live near a site where toxic waste is dumped. Additionally, some studies have found links between the risk of PBC and frequent use of nail polish.

• #27

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

  Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune disease with a progressive course that may extend over many decades. PBC is thought to be caused by a combination of genetic predisposition and environmental triggers, and is most commonly recognized in women in their 5th or 6th decade of life. […] Although there is no absolute certainty as to what causes PBC, the most widely accepted theory is that a genetically susceptible patient comes into contact with an autoimmune triggering event. This triggering event could be an environmental factor, virus, allergen, chemical or medication. There is no one trigger and every patient has their own immune triggering event. […] Genetic factors include MHC class II (DR8, DQA1*0102, DQ/1*0402), MHC class III (C4 null,c4B2), and non-MHC genes (Exon 1 of CTLA-4). Familial factors have also been identified, including PBC/positive AMA and impaired T-cell regulation, extrahepatic autoimmune diseases and a higher prevalence in people with a family member, especially an identical twin, with PBC. […] Potential environmental factors, especially in genetically vulnerable individuals, include recurrent urinary tract infections (potentially related to exposure to bacterial components or the antibiotic use), exposure to toxic chemicals and cigarette smoking.

• #28 Primary Biliary Cirrhosis | Liver Disease | Henry Ford Health – Detroit, MI

  https://www.henryford.com/services/digestive/liver-disease/treatments/biliary-cirrhosis

  Primary biliary cirrhosis is a serious, chronic inflammation of the liver that can lead to cirrhosis (liver scarring) and gradual destruction of the bile ducts, the tubes that transport bile from the liver. […] The exact cause of PBC is unknown, but it is thought to be an autoimmune disease, where the immune system mistakenly starts attacking healthy organs and tissues. […] Some researchers think bacterial, fungal or parasitic infections could be a possible trigger for PBC.

• #29 Primary biliary cholangitis – Wikipedia

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

  A genetic predisposition to disease has been thought to be important for some time. […] An environmental Gram-negative Alphaproteobacterium Novosphingobium aromaticivorans has been associated with this disease, with several reports suggesting an aetiological role for this organism. […] A failure of immune tolerance against the mitochondrial pyruvate dehydrogenase complex (PDC-E2) is a primary cause, with shedding of the antigen into apoptotic bodies or „apotopes” leading to the anatomic localization.

• #30 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

  PBC is associated with other autoimmune disorders, such as systemic sclerosis, Sjgren syndrome, CREST syndrome (also known as limited scleroderma), and autoimmune thyroiditis. […] T cells attack the small bile ducts. CD4 and CD8 T lymphocytes directly target biliary epithelial cells. The trigger for the immunologic attack on bile ducts is unknown. Exposure to foreign antigens, such as an infectious (bacterial or viral) or toxic agent, may be the instigating event. These foreign antigens might be structurally similar to endogenous proteins (molecular mimicry); then the subsequent immunologic reaction would be autoimmune and self-perpetuating. Destruction and loss of bile ducts lead to impaired bile formation and secretion (cholestasis). […] Chronic cholestasis thus leads to liver cell inflammation and scarring in the periportal areas. Eventually, hepatic inflammation decreases as hepatic fibrosis progresses to cirrhosis.

• #31 What Causes Primary Biliary Cholangitis (Formerly Primary Biliary Cirrhosis)? | myPBCteam

  https://www.mypbcteam.com/resources/what-causes-primary-biliary-cholangitis-formerly-primary-biliary-cirrhosis

  Some PBC risk factors are present when you’re born. These inherited factors generally can’t be changed. […] PBC isn’t a traditional type of genetic disease caused by a single mutation (gene change), but genetic mutations passed down within families may contribute to the condition. Researchers are still learning about what these gene changes are and why they lead to PBC. […] Living with an infection may increase your risk of developing PBC later on. For example, there’s a strong link between PBC and urinary tract infections. […] Although it’s not entirely clear why, researchers have found that some people with PBC have antibodies in their blood that target proteins found on bacteria or viruses. These antibodies, which may be left over from an infection, could mistakenly attack normal proteins that look similar to bacterial or viral proteins. This may lead to the liver inflammation seen in PBC.

• #32 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

  PBC is associated with other autoimmune disorders, such as systemic sclerosis, Sjgren syndrome, CREST syndrome (also known as limited scleroderma), and autoimmune thyroiditis. […] T cells attack the small bile ducts. CD4 and CD8 T lymphocytes directly target biliary epithelial cells. The trigger for the immunologic attack on bile ducts is unknown. Exposure to foreign antigens, such as an infectious (bacterial or viral) or toxic agent, may be the instigating event. These foreign antigens might be structurally similar to endogenous proteins (molecular mimicry); then the subsequent immunologic reaction would be autoimmune and self-perpetuating. Destruction and loss of bile ducts lead to impaired bile formation and secretion (cholestasis). […] Chronic cholestasis thus leads to liver cell inflammation and scarring in the periportal areas. Eventually, hepatic inflammation decreases as hepatic fibrosis progresses to cirrhosis.

• #33 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

  Primary biliary cholangitis (PBC) probably results from an autoimmune reaction. […] The cause is not clear but is probably an autoimmune reaction (in which the immune system attacks the bodys own tissues). Primary biliary cholangitis often occurs in people with other autoimmune disorders, such as rheumatoid arthritis, scleroderma, Sjgren syndrome, or autoimmune thyroiditis. […] An autoimmune cause is also thought possible because more than 95% of people with PBC have certain abnormal antibodies in their blood. These antibodies attack mitochondria (tiny structures that produce energy in cells). However, these antibodies are not involved in the destruction of the bile ducts. Other immune cells attack the bile ducts. What triggers this attack is unknown, but it may be exposure to a virus or a toxic substance.

• #34 Primary biliary cholangitis – Autoimmune Association

  https://autoimmune.org/disease-information/primary-biliary-cirrhosis/

  Primary Biliary Cholangitis is irritation and swelling (inflammation) of the bile ducts of the liver. […] The cause of inflamed bile ducts in the liver is not known. However, Primary Biliary Cholangitis is an autoimmune disorder. […] The disease may be linked to autoimmune disorders such as: celiac disease, Raynaud’s phenomenon, sicca syndrome (dry eyes or mouth), and thyroid disease.

• #35 Primary biliary cholangitis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000282.htm

  The cause of inflamed bile ducts in the liver is not known. However, primary biliary cholangitis is an autoimmune disorder. That means your body’s immune system mistakenly attacks healthy tissue. […] The disease may be linked to autoimmune disorders such as: Celiac disease, Raynaud phenomenon, Sicca syndrome (dry eyes or mouth), Thyroid disease. […] The disease most often affects middle-aged women. […] Primary biliary cirrhosis (the old name for the disease); PBC.

• #36 Causes of primary biliary cholangitis – British Liver Trust

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

  One of the first things people think of as a cause of liver disease is drinking alcohol. But this is not always the case. Primary biliary cholangitis (PBC) is not related to alcohol in any way. […] Doctors dont know exactly what causes primary biliary cholangitis. But we do know that its an autoimmune condition. […] In PBC, there seems to be some genetic link. Close relatives of someone with PBC have roughly 10 times the average risk of developing it themselves. […] In people who do have a genetic risk of PBC, its likely that the disease is triggered by something theyve come into contact with in the environment. For example, an infection, tobacco smoke or contact with some types of chemicals, including those in nail varnish and hair spray. […] PBC is an autoimmune condition where the immune system attacks the cells of the bile ducts in the liver. […] Autoimmune hepatitis (AIH) is another autoimmune disease of the liver. The immune system attacks liver cells (rather than the cells of the bile ducts, as happens in PBC). […] Around 1 in 10 people with PBC also have some degree of AIH. Your doctor will be able to tell this from your test results.

• #37 Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-diagnosis-and-prognosis-of-primary-biliary-cholangitis

  Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis and eventually may result in cirrhosis and liver failure. […] The terminology was changed from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disorder and its natural history. […] The pathogenesis of PBC, the treatment of PBC, and the treatment of pruritus due to cholestasis are discussed separately. […] Primary biliary cholangitis (PBC) is rare, with a reported prevalence of 19 to 402 cases per million persons. […] The vast majority of patients (90 to 95 percent) are women, and most patients are diagnosed between the ages of 30 and 65 years.

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

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

  Primary biliary cirrhosis (PBC) is a progressive disease of the liver caused by a buildup of bile within the liver (cholestasis) that results in damage to the small bile ducts that drain bile from the liver. […] The cause of PBC is unknown. It is most likely an autoimmune disease, where the body’s immune system attacks its cells. In this disease, the bile ducts are under attack and are destroyed. […] There may be a genetic component to the development of PBC since a person with the disease is likely to have a family member also afflicted. […] Women are nine times more likely than men to develop PBC. It most often develops in middle age between the ages of 40 to 60. […] The disease is seen more often in white, northern Europeans, compared to African-Americans. […] Other autoimmune diseases: People with other autoimmune diseases, such as rheumatoid arthritis or Sjgren’s syndrome, are at a higher risk of developing PBC. […] Environmental factors: Exposure to certain chemicals or infections may increase a person’s risk of developing PBC.

• #39 Rare and Serious Liver Diseases – Intercept Pharmaceuticals

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

  PBC is a rare autoimmune liver disease. […] 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. […] When bile is not able to move through the bile ducts, it collects in the liver and causes damage. […] PBC is progressive, which means that the damage gets worse over time. […] 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. […] It is estimated that 90% of people who are diagnosed with PBC are women. […] Many people who are diagnosed with PBC are between 40-60 years of age. […] Doctors can test for PBC by taking blood samples and measuring certain chemicals related to liver function. […] The tests will show if there are elevated levels of alkaline phosphatase (ALP) and if antimitochondrial antibodies (AMAs) are present, which can be indicators of PBC.

• #40

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

  Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune disease with a progressive course that may extend over many decades. PBC is thought to be caused by a combination of genetic predisposition and environmental triggers, and is most commonly recognized in women in their 5th or 6th decade of life. […] Although there is no absolute certainty as to what causes PBC, the most widely accepted theory is that a genetically susceptible patient comes into contact with an autoimmune triggering event. This triggering event could be an environmental factor, virus, allergen, chemical or medication. There is no one trigger and every patient has their own immune triggering event. […] Genetic factors include MHC class II (DR8, DQA1*0102, DQ/1*0402), MHC class III (C4 null,c4B2), and non-MHC genes (Exon 1 of CTLA-4). Familial factors have also been identified, including PBC/positive AMA and impaired T-cell regulation, extrahepatic autoimmune diseases and a higher prevalence in people with a family member, especially an identical twin, with PBC. […] Potential environmental factors, especially in genetically vulnerable individuals, include recurrent urinary tract infections (potentially related to exposure to bacterial components or the antibiotic use), exposure to toxic chemicals and cigarette smoking.

• #41 Primary biliary cirrhosis | Better Health Channel

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

  Primary biliary cholangitis (previously known as primary biliary cirrhosis) is characterised by the chronic inflammation and scarring of the bile ducts within the liver. […] For reasons unknown, the immune system attacks the cells that line the bile ducts. […] The cause is unknown, although researchers have ruled out alcohol and diet as possible triggers. […] PBC is an autoimmune disorder. The immune system attacks the bile ducts, but the trigger for this attack is so far unidentified. Alcohol and diet are not thought to play significant roles in the development of the disease. […] PBC seems to be slightly more common in families, which suggests a genetic susceptibility. […] For reasons unknown, the disease seems to be more common in Scotland, Scandinavia and North East England.

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

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

  Primary biliary cirrhosis (PBC) is a progressive disease of the liver caused by a buildup of bile within the liver (cholestasis) that results in damage to the small bile ducts that drain bile from the liver. […] The cause of PBC is unknown. It is most likely an autoimmune disease, where the body’s immune system attacks its cells. In this disease, the bile ducts are under attack and are destroyed. […] There may be a genetic component to the development of PBC since a person with the disease is likely to have a family member also afflicted. […] Women are nine times more likely than men to develop PBC. It most often develops in middle age between the ages of 40 to 60. […] The disease is seen more often in white, northern Europeans, compared to African-Americans. […] Other autoimmune diseases: People with other autoimmune diseases, such as rheumatoid arthritis or Sjgren’s syndrome, are at a higher risk of developing PBC. […] Environmental factors: Exposure to certain chemicals or infections may increase a person’s risk of developing PBC.

• #43 Pathogenesis of primary biliary cholangitis (primary biliary cirrhosis) – UpToDate

  https://www.uptodate.com/contents/pathogenesis-of-primary-biliary-cholangitis-primary-biliary-cirrhosis

  Pathogenesis of primary biliary cholangitis (primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis, and eventually results in cirrhosis and liver failure. […] The precise cause of PBC is unknown but, as with other autoimmune diseases, is related to genetic susceptibility and environmental factors. A number of environmental causes have been implicated, including several bacteria, viruses, toxins, and drugs. Some of the most compelling evidence for an environmental factor has been derived from epidemiologic studies, which have demonstrated geographic clustering, clustering of cases across time, and seasonal variation in the diagnosis of PBC.

• #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. […] It’s not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors. […] The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver. […] Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

• #45 Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-diagnosis-and-prognosis-of-primary-biliary-cholangitis

  Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis and eventually may result in cirrhosis and liver failure. […] The terminology was changed from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disorder and its natural history. […] The pathogenesis of PBC, the treatment of PBC, and the treatment of pruritus due to cholestasis are discussed separately. […] Primary biliary cholangitis (PBC) is rare, with a reported prevalence of 19 to 402 cases per million persons. […] The vast majority of patients (90 to 95 percent) are women, and most patients are diagnosed between the ages of 30 and 65 years.

• #46 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

  PBC is associated with other autoimmune disorders, such as systemic sclerosis, Sjgren syndrome, CREST syndrome (also known as limited scleroderma), and autoimmune thyroiditis. […] T cells attack the small bile ducts. CD4 and CD8 T lymphocytes directly target biliary epithelial cells. The trigger for the immunologic attack on bile ducts is unknown. Exposure to foreign antigens, such as an infectious (bacterial or viral) or toxic agent, may be the instigating event. These foreign antigens might be structurally similar to endogenous proteins (molecular mimicry); then the subsequent immunologic reaction would be autoimmune and self-perpetuating. Destruction and loss of bile ducts lead to impaired bile formation and secretion (cholestasis). […] Chronic cholestasis thus leads to liver cell inflammation and scarring in the periportal areas. Eventually, hepatic inflammation decreases as hepatic fibrosis progresses to cirrhosis.

• #47 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. […] It’s not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors. […] The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver. […] Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

• #48 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

  PBC is associated with other autoimmune disorders, such as systemic sclerosis, Sjgren syndrome, CREST syndrome (also known as limited scleroderma), and autoimmune thyroiditis. […] T cells attack the small bile ducts. CD4 and CD8 T lymphocytes directly target biliary epithelial cells. The trigger for the immunologic attack on bile ducts is unknown. Exposure to foreign antigens, such as an infectious (bacterial or viral) or toxic agent, may be the instigating event. These foreign antigens might be structurally similar to endogenous proteins (molecular mimicry); then the subsequent immunologic reaction would be autoimmune and self-perpetuating. Destruction and loss of bile ducts lead to impaired bile formation and secretion (cholestasis). […] Chronic cholestasis thus leads to liver cell inflammation and scarring in the periportal areas. Eventually, hepatic inflammation decreases as hepatic fibrosis progresses to cirrhosis.

• #49 Rare and Serious Liver Diseases – Intercept Pharmaceuticals

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

  PBC is a rare autoimmune liver disease. […] 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. […] When bile is not able to move through the bile ducts, it collects in the liver and causes damage. […] PBC is progressive, which means that the damage gets worse over time. […] 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. […] It is estimated that 90% of people who are diagnosed with PBC are women. […] Many people who are diagnosed with PBC are between 40-60 years of age. […] Doctors can test for PBC by taking blood samples and measuring certain chemicals related to liver function. […] The tests will show if there are elevated levels of alkaline phosphatase (ALP) and if antimitochondrial antibodies (AMAs) are present, which can be indicators of PBC.

• #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. […] It’s not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors. […] The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver. […] Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they’re replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

• #51 Pathophysiology of biochemical signs of primary biliary cholangitis

  https://www.explorationpub.com/Journals/edd/Article/100524

  Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a rare chronic autoimmune cholestatic liver disease, affecting mostly females. […] Defects in autoimmune tolerance are critical factors in the emergence of the disease. […] PBC develops due to a defect in autoimmune tolerance. […] It is precisely these changes in the processes of bile formation and excretion in patients with PBC that should be considered as the underlying cause of biochemical signs of this disease. […] There is a change in many biochemical parameters of blood in patients with PB. […] The mechanism of the increase in ALP and 5-NT levels in PBC is the subject of debate. […] The entrance of bile acids into the blood plasma of patients with PBC is one of the causes of the development of pruritus already in the asymptomatic stage of the disease and lipid metabolism disorders.

• #52 Pathophysiology of biochemical signs of primary biliary cholangitis

  https://www.explorationpub.com/Journals/edd/Article/100524

  Dyslipidemia is detected in patients with PB already in an early stage of the disease. […] The increase in the level of TC and LDL-C in patients with PBC is alarming for many authors, since these indicators are considered as risk factors for the development of cardiovascular events. […] However, hypercholesterolemia developing with cholestasis is aimed at neutralizing the detergent properties of bile acids entering the systemic circulation. […] The development of liver cirrhosis at a late stage of PBC is accompanied by a disturbance of the protein-synthesizing function of liver cells. […] The imbalance associated with the lower concentration of the vitamin K-dependent glycoproteins C and S than that of the decreased level of blood clotting factors may contribute to increased thrombosis.

• #53 About PBC | PBC Foundation

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

  PBC is an autoimmune liver condition. […] PBC IS AN AUTOIMMUNE DISEASE THAT AFFECTS THE LIVER. […] Primary Biliary Cholangitis, known for short as PBC, is a lifelong, autoimmune condition which affects the liver. […] The bile acids then leak from the bile ducts, damaging surrounding liver cells, which then causes inflammation and scarring in the liver. […] This widespread damage and scarring may result in cirrhosis, however most people with PBC will never develop cirrhosis. […] PBC was formerly known as Primary Biliary Cirrhosis. […] Whilst we know that two aspects need to be in place for someone to develop PBC: a genetic element (predisposition) and an environmental trigger, we do not know what causes PBC or what can cure PBC. […] There is a slightly increased risk of your first-degree relatives (especially mother/daughter) having PBC. […] But the current reality is that most patients will die with their PBC and not because of their PBC.

• #54

  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. […] In PBC, the immune system (the body’s natural defence against infection and illness) mistakenly attacks the bile ducts. […] It’s not clear why this happens, but it’s thought to be caused by a combination of subtle differences in how the immune system works. […] The bile ducts become damaged and injured, causing bile to build up in the liver. This further damages the liver and may lead to scarring (cirrhosis).

• #55 Pathophysiology of biochemical signs of primary biliary cholangitis

  https://www.explorationpub.com/Journals/edd/Article/100524

  Dyslipidemia is detected in patients with PB already in an early stage of the disease. […] The increase in the level of TC and LDL-C in patients with PBC is alarming for many authors, since these indicators are considered as risk factors for the development of cardiovascular events. […] However, hypercholesterolemia developing with cholestasis is aimed at neutralizing the detergent properties of bile acids entering the systemic circulation. […] The development of liver cirrhosis at a late stage of PBC is accompanied by a disturbance of the protein-synthesizing function of liver cells. […] The imbalance associated with the lower concentration of the vitamin K-dependent glycoproteins C and S than that of the decreased level of blood clotting factors may contribute to increased thrombosis.

• #56 About PBC | PBC Foundation

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

  PBC is an autoimmune liver condition. […] PBC IS AN AUTOIMMUNE DISEASE THAT AFFECTS THE LIVER. […] Primary Biliary Cholangitis, known for short as PBC, is a lifelong, autoimmune condition which affects the liver. […] The bile acids then leak from the bile ducts, damaging surrounding liver cells, which then causes inflammation and scarring in the liver. […] This widespread damage and scarring may result in cirrhosis, however most people with PBC will never develop cirrhosis. […] PBC was formerly known as Primary Biliary Cirrhosis. […] Whilst we know that two aspects need to be in place for someone to develop PBC: a genetic element (predisposition) and an environmental trigger, we do not know what causes PBC or what can cure PBC. […] There is a slightly increased risk of your first-degree relatives (especially mother/daughter) having PBC. […] But the current reality is that most patients will die with their PBC and not because of their PBC.

• #57 Video on the Liver Disease Primary Biliary Cholangitis

  https://www.webmd.com/digestive-disorders/video/video-primary-biliary-cholangitis

  Primary billiary cholangitis, or PBC, is a chronic disease that gradually damages the bile ducts, and eventually your liver. You may also hear it called primary billiary cirrhosis. PBC is thought to be a problem with your body’s immune system. When you have it, your body mistakenly attacks your bile ducts. It causes inflammation and swelling that, over time, can lead to a buildup of bile in your liver. That can result in damage and scarring. PBC can lead to liver failure. […] While there’s no cure for PBC, early detection and treatment can slow its progression.

• #58 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.

• #59 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.

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

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

  Our study revealed that 24.2% of patients with precirrhotic PBC may experience PH. According to Nakanuma’s pathologic staging, there were 5.2% (1/19) of patients with PH in the histologic stage I of our study, 17.3% (17/98) of the patients in stage II had PH, and 45.8% (22/48) of the patients in stage III had comorbid PH. The occurrence of combined PH increases as the histology progresses. […] In summary, the percentage of precirrhotic PBC patients with PH at the time of diagnosis was 24.2% (40/165). High ALP decreased PLT count, high Mayo score, and high FIB-4 index at the time of diagnosis were effective predictors of the presence of PH in precirrhotic PBC patients, and tests related to PH should be reviewed regularly in such patients. The presence of combined PH in precirrhotic of PBC is associated with cholestasis. Cholestasis due to bile duct defects may affect the function of endothelial cells in portal vein vessels, which in turn may lead to venous hypertension.

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

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

  Our study revealed that 24.2% of patients with precirrhotic PBC may experience PH. According to Nakanuma’s pathologic staging, there were 5.2% (1/19) of patients with PH in the histologic stage I of our study, 17.3% (17/98) of the patients in stage II had PH, and 45.8% (22/48) of the patients in stage III had comorbid PH. The occurrence of combined PH increases as the histology progresses. […] In summary, the percentage of precirrhotic PBC patients with PH at the time of diagnosis was 24.2% (40/165). High ALP decreased PLT count, high Mayo score, and high FIB-4 index at the time of diagnosis were effective predictors of the presence of PH in precirrhotic PBC patients, and tests related to PH should be reviewed regularly in such patients. The presence of combined PH in precirrhotic of PBC is associated with cholestasis. Cholestasis due to bile duct defects may affect the function of endothelial cells in portal vein vessels, which in turn may lead to venous hypertension.

• #62 Primary Biliary Cholangitis (Primary Biliary Cirrhosis) – NIDDK

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

  Primary biliary cholangitis is a chronic disease in which the small bile ducts in the liver become injured and inflamed and are eventually destroyed. […] Experts arent sure what causes primary biliary cholangitis, but genes and environmental triggers may play a role.

• #63 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. […] Doctors dont know exactly what causes this immune system attack. Its likely triggered by both genetic and environmental causes. […] PBC is an autoimmune disease. This means your immune system mistakes tissue in your liver for a foreign invader and attacks it.

• #64 Primary Biliary Cholangitis – StatPearls – NCBI Bookshelf

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

  Primary biliary cholangitis (PBC), formerly termed primary biliary cirrhosis, is an autoimmune disorder that gradually destroys intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. […] Researchers theorize that patient with primary biliary cirrhosis has both genetic predispositions and the right environmental trigger. The prevalence of the disease is 100-fold higher in first-degree relatives of the index patient, which strongly suggests a genetic predisposition. […] The inflammation is thought to result from a direct insult of environmental factors and toxins. […] The pathogenesis of primary biliary cholangitis is thought to be related to the interaction between genetic predisposition and environmental triggers. […] The genetic predisposition is suggested by a strong prevalence of the disease in first-degree relatives, with an odds ratio of 11.

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

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

  If you have primary biliary cholangitis (PBC), it means the bile ducts in your liver a series of tubes that transport bile through your liver, small intestine and gallbladder are damaged. […] Its believed that an overactive immune system causes primary biliary cholangitis, but what triggers this over-activity still isnt clearly known. […] A genetic predisposition for PBC likely exists, and exposure to certain bacteria, viruses or toxins is believed to somehow trigger the immune system, which reacts in an inappropriate way and causes injury to the bile ducts.

• #66 Primary biliary cirrhosis | Better Health Channel

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

  Primary biliary cholangitis (previously known as primary biliary cirrhosis) is characterised by the chronic inflammation and scarring of the bile ducts within the liver. […] For reasons unknown, the immune system attacks the cells that line the bile ducts. […] The cause is unknown, although researchers have ruled out alcohol and diet as possible triggers. […] PBC is an autoimmune disorder. The immune system attacks the bile ducts, but the trigger for this attack is so far unidentified. Alcohol and diet are not thought to play significant roles in the development of the disease. […] PBC seems to be slightly more common in families, which suggests a genetic susceptibility. […] For reasons unknown, the disease seems to be more common in Scotland, Scandinavia and North East England.

• #67 Causes of primary biliary cholangitis – British Liver Trust

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

  One of the first things people think of as a cause of liver disease is drinking alcohol. But this is not always the case. Primary biliary cholangitis (PBC) is not related to alcohol in any way. […] Doctors dont know exactly what causes primary biliary cholangitis. But we do know that its an autoimmune condition. […] In PBC, there seems to be some genetic link. Close relatives of someone with PBC have roughly 10 times the average risk of developing it themselves. […] In people who do have a genetic risk of PBC, its likely that the disease is triggered by something theyve come into contact with in the environment. For example, an infection, tobacco smoke or contact with some types of chemicals, including those in nail varnish and hair spray. […] PBC is an autoimmune condition where the immune system attacks the cells of the bile ducts in the liver. […] Autoimmune hepatitis (AIH) is another autoimmune disease of the liver. The immune system attacks liver cells (rather than the cells of the bile ducts, as happens in PBC). […] Around 1 in 10 people with PBC also have some degree of AIH. Your doctor will be able to tell this from your test results.

• #68 Primary Biliary Cirrhosis & Cholangitis (PBC): Symptoms, Causes & Diagnosis

  https://www.emedicinehealth.com/primary_biliary_cirrhosis/article_em.htm

  The possibility that PBC is caused by an infection with a virus, bacterium, or fungus has generated several studies. […] Investigators are currently pursuing leads suggesting that the biliary epithelial cells of individuals with PBC may contain an infectious virus that belongs to the class of viruses called retroviruses. […] PBC is not transmitted by heredity from parents with the disease to their children. Thus, PBC is not a classical hereditary (genetic) disease, as is diabetes, for example. […] Studies have shown that there are some weak associations between PBC and certain specific inherited genes of the immune system.

• #69 What is PBC – PBC Society Canada

  https://pbc-society.ca/what-is/

  Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is a slowly progressive bile duct disease, which results from chronic inflammation of the small intrahepatic bile ducts, the system of small tubes in the liver that helps drain bile. […] It is thought to be caused by a persons immune system attacking his or her own liver, but the cause is still unknown. […] At present the cause is unknown. It is not caused by alcohol or any known transmissible diseases. PBC is thought to have an autoimmune basis, probably triggered by an environmental factor in a person with a subtle genetic predisposition. […] Researcher have identified genes that are associated with the development of PBC. These genes are also implicated in related autoimmune diseases, often seen in patients and their families, e.g. celiac disease, rheumatoid arthritis, scleroderma. […] Other studies are being performed to look at the possibility of environmental triggers of PBC, such as bacteria, viruses and chemicals. No environmental agents have been confirmed to date.

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

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

  Ursodeoxycholic acid (UDCA) is the most common medicine used, with the aim of slowing the progression of the disease. […] Immunosuppressive medicines are sometimes used, including penicillamine, azathioprine, methotrexate, ciclosporin and steroids. […] Various other autoimmune diseases are more common in people with PBC. […] A liver transplant is an option if the liver becomes badly damaged due to cirrhosis. […] PBC is a progressive disease. Most commonly it progresses very slowly over a number of years.

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