Materiały źródłowe

• #1 Primary Sclerosing Cholangitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537181/

  Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. […] The etiology of PSC is not understood; however, genetic and environmental factors contribute to its development. […] Inflammatory bowel disease (IBD) is considered to be a significant risk factor for the development of PSC. […] PSC is believed to be an autoimmune disorder. […] In addition, individuals with HLA B8 and HLA DR3 genes are at a higher risk of developing the disorder.

• #2 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology

  https://tgh.amegroups.org/article/view/6491/html

  Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease characterized by inflammatory destruction of the intrahepatic and/or extrahepatic bile ducts, leading to bile stasis, fibrosis, and ultimately to cirrhosis, and often requires liver transplantation (LT). […] The etiology of PSC is poorly understood, but an increasing body of evidence supports the concept of cholangiocyte injury as a result of environmental exposure and an abnormal immune response in genetically susceptible individuals. […] The pathogenesis of PSC is not fully understood but appears to be multifactorial and several mechanistic theories have been proposed. Cholangiocyte injury appears to result from environmental exposure and an abnormal cholangiocyte immune response leading to clinical disease in genetically susceptible individuals.

• #3 Primary Sclerosing Cholangitis: Background, Etiopathophysiology, Epidemiology

  https://emedicine.medscape.com/article/931355-overview

  The etiology of primary sclerosing cholangitis (PSC) remains unknown, but it is thought to be multifactorial, including genetic predisposition, exposure to an environmental antigen, and subsequent aberrant immunologic response to that stimulus. […] There is also an increased prevalence of HLA alleles A1, B8, and DR3 in PSC. […] An autoimmune mechanism is suggested, because approximately 70% of patients with PSC have inflammatory bowel disease (IBD). […] A marked increase in serum autoantibody levels occurs in patients with PSC as well, with antineutrophil cytoplasmic antibodies (ANCA) in 87%, anticardiolipin (aCL) antibodies in 66%, and antinuclear antibodies (ANA) in 53%. […] In the biliary ducts, an inflammatory response to chronic or recurrent bacterial infection in the portal circulation and from exposure to toxic bile acids has been postulated.

• #4 Primary sclerosing cholangitis – Rabiee – Translational Gastroenterology and Hepatology

  https://tgh.amegroups.org/article/view/6491/html

  The role of genetic factors in the etiology of PSC is underscored by the finding that first-degree relatives of patients with PSC have an increased risk of PSC (up to 11-fold). […] Genetic susceptibility factors for PSC may overlap with UC as first-degree relatives of patients with PSC without IBD are also at an increased risk of UC (8-fold). […] Nonetheless, genetic findings in PSC so far explain less than 10% of disease liability, and environmental risk factors are estimated to account for greater than 50% of the unexplained fraction.

• #5 Primary sclerosing cholangitis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/primary-sclerosing-cholangitis/

  Primary sclerosing cholangitis is thought to arise from a combination of genetic and environmental factors. Researchers believe that genetic changes play a role in this condition because it often occurs in several members of a family and because immediate family members of someone with primary sclerosing cholangitis have an increased risk of developing the condition. […] It is likely that specific genetic variations increase a person’s risk of developing primary sclerosing cholangitis, and then exposure to certain environmental factors triggers the disorder. However, the genetic changes that increase susceptibility and the environmental triggers remain unclear. […] There is evidence that variations in certain genes involved in immune function influence the risk of developing primary sclerosing cholangitis. The most commonly associated genes belong to a family of genes called the human leukocyte antigen (HLA) complex.

• #6 Primary sclerosing cholangitis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/primary-sclerosing-cholangitis/

  Specific variations of several HLA genes seem to be present more often in people with primary sclerosing cholangitis than in people who do not have the disorder. These variations may dysregulate the body’s immune response, leading to the inflammation of the bile ducts in people with primary sclerosing cholangitis. However, the mechanism is not well understood. […] The inheritance pattern of primary sclerosing cholangitis is unknown because many genetic and environmental factors are likely to be involved. This condition tends to cluster in families, however, and having an affected family member is a risk factor for developing the disease.

• #7 Primary sclerosing cholangitis – Wikipedia

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

  Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. […] The underlying cause of PSC is unknown. Genetic susceptibility, immune system dysfunction, and abnormal composition of the gut flora may play a role. […] The exact cause of primary sclerosing cholangitis is unknown, and its pathogenesis is improperly understood. Although PSC is thought to be caused by autoimmune disease, it does not demonstrate a clear response to immunosuppressants. Thus, many experts believe it to be a complex, multifactorial (including immune-mediated) disorder and perhaps one that encompasses several different hepatobiliary diseases. […] Data have provided novel insights suggesting: an important association between the intestinal microbiota and PSC and a process referred to as cellular senescence and the senescence-associated secretory phenotype in the pathogenesis of PSC. […] In addition, longstanding, well-recognized associations are seen between PSC and human leukocyte antigen alleles (A1, B8, and DR3).

• #8 Primary Sclerosing Cholangitis: Background, Etiopathophysiology, Epidemiology

  https://emedicine.medscape.com/article/931355-overview

  A genetic predisposition has been suggested because of an increased prevalence of HLA-B8, HLA-DR3, and HLA-Drw52a. […] Genome-wide association studies (GWAS) performed in PSC have identified about 20 genes that are significantly associated with PSC, most of which localize within the human leukocyte antigen (HLA) complex. […] The most plausible concept of the pathogenesis of PSC involves the exposure of genetically predisposed individuals to an environmental antigen that subsequently elicits an aberrant immune response, leading to development of the disease.

• #9

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  More than 20 risk genes predominantly within the HLA complex or associated with IBD and other immune-mediated diseases were identified in PSC patients in a large genome-wide association study. […] The role of T-cells (Th17) has been confirmed in PSC-IBD patients in whom specific intestinal bacteria-induced pore formation in the intestinal epithelium and subsequent bacterial translocation with the immune-inflammatory response of the hepatobiliary tract. […] Together with the recent finding of alteration in intestinal fungi composition, this reinforces the long-considered hypothesis of a causal role for gut microbiota dysbiosis in the pathogenesis of PSC. […] The main mechanisms of cholangiocyte protection against the potentially harmful effects of biliary bile acid monomers are apparently the secretion of bicarbonate with the formation of a biliary bicarbonate umbrella, an alkaline layer on the apical membrane of cholangiocytes stabilized by the biliary glycocalyx, and the luminal formation of mixed micelles of phospholipids, cholesterol and bile acids.

• #10

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10-15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. […] Primary sclerosing cholangitis (PSC) is a chronic cholestatic hepatobiliary disease of unknown etiology affecting both intra- and extrahepatic bile ducts. […] Genetic factors contribute about 10% to a predisposition for PSC and may explain the increased risk of PSC in first-degree relatives of PSC patients.

• #11 Primary Sclerosing Cholangitis (PSC) – Hepatic and Biliary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/primary-sclerosing-cholangitis-psc

  Primary sclerosing cholangitis (PSC) is patchy inflammation, fibrosis, and strictures of the bile ducts that has no known cause. However, 80% of patients with PSC also have inflammatory bowel disease, most often ulcerative colitis. […] Although the cause is unknown, primary sclerosing cholangitis (PSC) is associated with inflammatory bowel disease, which is present in 70% of patients. About 5% of patients with ulcerative colitis and about 1% with Crohn disease develop primary sclerosing cholangitis (PSC). This association and the presence of several autoantibodies (eg, antinuclear antibodies [ANA] and perinuclear antineutrophilic antibodies [pANCA]) suggest immune-mediated mechanisms. T cells appear to be involved in the destruction of the bile ducts, implying disordered cellular immunity. A genetic predisposition is suggested by a tendency for the disorder to develop in multiple family members and a higher frequency in people with HLAB8 and HLADR3, which are often correlated with autoimmune disorders. An unknown trigger (eg, bacterial infection, ischemic duct injury) presumably precipitates development of PSC in genetically predisposed people.

• #12 Primary sclerosing cholangitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/primary-sclerosing-cholangitis?lang=us

  Primary sclerosing cholangitis (PSC) is an uncommon inflammatory condition that affects the biliary tree resulting in multiple strictures, liver damage, and eventually cirrhosis. […] Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease (IBD) (in 70% of cases), especially ulcerative colitis, and thus shares similar demographics: young to middle-aged males (~4th decade) are most frequently affected. […] 70-80% of patients with primary sclerosing cholangitis develop inflammatory bowel disease. […] Most patients demonstrate at least one type of autoantibody, typically anti-smooth muscle antibody (ASMA) and antinuclear antibody (ANA), which are seen in up to 75%. […] Primary sclerosing cholangitis is a risk factor for developing gallbladder cancer, so gallbladder polyps should be considered malignancy until proven otherwise.

• #13 :: KJR :: Korean Journal of Radiology

  https://www.kjronline.org/DOIx.php?id=10.3348/kjr.2016.17.1.25

  The diagnosis of PSC requires the exclusion of secondary causes of sclerosing cholangitis. […] The prevalence of PSC is approximately 10/100000 in Northern Europe and the USA, while it is far less common in Southern Europe and Asia. […] PSC has a strong association with inflammatory bowel diseases. Approximately 60 to 80% of patients with PSC present with inflammatory bowel disease of which, 87% have ulcerative colitis and 13% have Crohn’s disease. […] Medical therapy such as the use of ursodeoxycholic acid, corticosteroids or other immunosuppressive agents have shown only limited success in patients with PSC. […] In patients with advanced liver disease, liver transplantation is recommended as a curative treatment option. […] Recurrence of PSC after liver transplantation occurs in 20 to 25% of patients 5 to 10 years after the liver transplantation.

• #14 Primary sclerosing cholangitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-41

  Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. […] PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. […] The aetiology and pathogenesis of PSC are still unknown. […] Lymphocytic portal tract infiltration and associations with human leukocyte antigen (HLA) haplotypes and autoantibodies are suggestive of an immune mediated basis for this disease. […] Autoimmune diseases are more frequent in patients with PSC than in patients with IBD who do not have PSC. […] PSC is probably acquired through inheriting a combination of genetic polymorphisms that interrelate to cause disease susceptibility. […] The association of PSC and IBD led to Vierling’s hypothesis that PSC may be triggered by infections or toxins acting as molecular mimics entering the portal circulation through a permeable colon.

• #15 Primary Sclerosing Cholangitis (PSC) — Irish Liver Foundation

  https://www.liverfoundation.ie/primary-sclerosing-cholangitis-psc

  The exact cause of PSC is unclear. […] Because of the strong association between PSC and IBD, there may be a common cause for both diseases. […] In theory, the inflamed colon/bowel could allow toxins or bacteria to enter the bloodstream which then track to the liver and cause inflammation and damage to the bile ducts.

• #16 Causes of Primary Sclerosing Cholangitis: The “Leaky Gut” Theory – Digestive Health Foundation

  https://digestivehealthfoundation.org/project/causes-of-primary-sclerosing-cholangitis-the-leaky-gut-theory/

  Primary sclerosing cholangitis (PSC) causes chronic inflammation of the bile ducts and, over time, potential liver failure. What triggers PSC and how it is strongly linked to ulcerative colitis (UC) remains unclear. One theory is that a leaky gut allows toxic compounds from the intestines to erroneously enter the liver. […] Researchers anticipate that the sugar levels in the urine will be different between patients with PSC, patients with PSC and ulcerative colitis, and healthy patients. If this occurs, the team will evaluate therapies, such as antibiotics, to assess their impact on the leaky gut.

• #17 Primary sclerosing cholangitis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/primary-sclerosing-cholangitis

  It’s not clear what causes primary sclerosing cholangitis. An immune system reaction to an infection or toxin may trigger the disease in people who are genetically predisposed to it. […] A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn’s disease. […] Primary sclerosing cholangitis and inflammatory bowel disease don’t always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. If primary sclerosing cholangitis is diagnosed, it’s important to look for inflammatory bowel disease because there is a greater risk of colon cancer. […] Somewhat less often, people being treated for inflammatory bowel disease turn out to have primary sclerosing cholangitis as well. And rarely, people with primary sclerosing cholangitis develop inflammatory bowel disease only after having a liver transplant.

• #18 Pulsenotes | PSC

  https://app.pulsenotes.com/medicine/hepatology/notes/primary-sclerosing-cholangitis

  Primary sclerosing cholangitis is an immune-mediated disease characterised by cholestasis, bile duct strictures and hepatic fibrosis. […] The exact cause of PSC remains unknown. […] PSC is considered a progressive autoimmune disorder that is strongly associated with inflammatory bowel disease (IBD). […] An immune-mediated reaction develops that causes bile duct damage. […] It is postulated that a chronic inflammatory response occurs in response to recurrent exposure to bacteria within the portal system, accumulation of toxic bile acids or ischaemic bile duct injury. […] Both Crohns disease and Ulcerative colitis are associated with PSC. […] The association between IBD and PSC depends on geographical locations. […] Treatment of IBD has no impact on the overall progression of PSC.

• #19 Primary sclerosing cholangitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/primary-sclerosing-cholangitis?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjgh&lang=gb

  Primary sclerosing cholangitis (PSC) is an uncommon inflammatory condition that affects the biliary tree resulting in multiple strictures, liver damage, and eventually cirrhosis. […] Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease (IBD) (in 70% of cases), especially ulcerative colitis, and thus shares similar demographics: young to middle-aged males (~4th decade) are most frequently affected. […] 70-80% of patients with primary sclerosing cholangitis develop inflammatory bowel disease. […] Most patients demonstrate at least one type of autoantibody, typically anti-smooth muscle antibody (ASMA) and antinuclear antibody (ANA), which are seen in up to 75%. […] p-ANCA is also positive in up to 80% of patients with primary sclerosing cholangitis. […] Primary sclerosing cholangitis is a risk factor for developing gallbladder cancer, so gallbladder polyps should be considered malignancy until proven otherwise.

• #20 Orphanet: Primary sclerosing cholangitis

  https://www.orpha.net/en/disease/detail/171

  Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure. […] The precise etiology of PSC has not yet been established, but it is believed to be multifactorial. Genetic susceptibility factors are known to be involved, and a strong association to genetic variants within the HLA-region on chromosome 6 is observed. The disease is thought to be related to an abnormal immune response to a still unidentified environmental stimulus.

• #21 Primary Sclerosing Cholangitis (PSC) | University Hospitals

  https://www.uhhospitals.org/health-information/health-and-wellness-library/article/diseases-and-conditions/primary-sclerosing-cholangitis-psc

  Primary sclerosing cholangitis (PSC) is a rare liver disease that causes the bile ducts inside and outside the liver to become inflamed and scarred. […] Nobody knows the exact cause of PSC. […] Some of the possible causes include: Genes. Having a family member with PSC increases your risk. […] Germs. Bacteria and viruses may play a role in setting off PSC. […] Inflammatory bowel disease. Many people with PSC also have ulcerative colitis, an inflammatory bowel disease. […] Immune system problems. Your immune system is your body’s defense system. An abnormal immune system may play a role in PSC.

• #22 Symptoms & Causes of Primary Sclerosing Cholangitis – NIDDK

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

  Experts arent sure what causes PSC. […] Studies suggest that several factors may play a role, including genes, immune system problems, changes in the bacteria in the digestive tract, also called gut flora or the gut microbiome, bile duct injury caused by bile acids.

• #23 Primary Sclerosing Cholangitis | The Oregon Clinic

  https://www.oregonclinic.com/resource/primary-sclerosing-cholangitis/

  Researchers do not know what causes PSC. […] Among the theories under investigation are the possible role of bacteria, viruses, and immune system problems. […] PSC most often appears to be associated with ulcerative colitis, a type of inflammatory bowel disease.

• #24 Primary Sclerosing Cholangitis (PSC) – Guts UK

  https://gutscharity.org.uk/advice-and-information/conditions/primary-sclerosing-cholangitis-psc/

  Primary Sclerosing Cholangitis (PSC) is a rare, immune related disease that affects bile ducts and liver. […] The cause of PSC is not fully known, but research suggests that PSC is an autoimmune disease. Doctors and researchers suggest that certain genetic factors interact with unknown environmental or lifestyle triggers, to make a person more prone to develop the disease. […] The strong association with IBD suggests that changes in the gut bacteria are involved in disease development. PSC is not caused by alcohol.

• #25

  https://link.springer.com/article/10.1007/s12072-020-10118-x

  It is hypothesized that stabilization of the biliary bicarbonate umbrella may slow down the progression of fibrosing cholangiopathies. […] A role of the bicarbonate umbrella in PSC pathogenesis is supported by the association of PSC with gene sequence variations of TGR5, a cholangiocellular bile acid receptor promoting chloride and bicarbonate secretion, and by downregulation of the TGR5 protein in cholangiocytes of PSC patients. […] Also associations with PSC of other gene variants encoding for stabilizers of the apical cholangiocyte membrane of cholangiocytes are suggestive of defects of the biliary bicarbonate umbrella in PSC. […] Intracellular accumulation of potentially toxic bile acids during cholestasis in humans contributes to hepatocyte and cholangiocyte damage, inflammation, and the development and progression of cholestatic diseases.

• #26 Primary Sclerosing Cholangitis | Liver Disease | Henry Ford Health – Detroit, MI

  https://www.henryford.com/services/digestive/liver-disease/treatments/sclerosing-cholangitis

  Primary sclerosing cholangitis (PSC) is a condition produced by inflammation and scarring of the bile ducts, the tubes that carry digestive fluid from your liver to your small intestine. […] Researchers dont yet know what causes primary sclerosing cholangitis (PSC). However, PSC risk factors include: […] A history of inflammatory bowel disease: Includes ulcerative colitis or Crohns disease. […] Age: PSC usually appears in young adults but may develop at any age. […] Gender: PSC is more common in men than women.

• #27 Primary Sclerosing Cholangitis (PSC) – Cirrhosis Care

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

  Primary sclerosing cholangitis (PSC) is a liver disease that causes inflammation and scarring in the bile ducts, making them shrink over time. […] This inflammation results from immune cells attacking the bile ducts. PSC is called primary because the cause is unknown, sclerosing because of the scarring, and cholangitis because the inflammation occurs in the bile ducts. […] The disease affects both sexes, but its more common in men. It occurs in people mostly between the ages of 30 and 50. […] There is no specific treatment for slowing the advance of PSC. Unfortunately, PSC is relentless over time. It leads to liver failure and the need for liver transplant with almost all patients.

• #28 Primary Sclerosing Cholangitis (PSC)

  https://liverfoundation.org/liver-diseases/autoimmune-liver-diseases/primary-sclerosing-cholangitis-psc/

  Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts. […] The cause of this disease is not known. About 70 percent of patients are men. It may be related to bacterial or viral infections, as well as problems in the immune system. Genetic factors may also play a role. PSC is considered an uncommon disease, but recent studies suggest that it may be more common than previously thought. […] PSC is slightly more common in men than women. It usually affects people between the ages of 30 and 60. As many as 75 percent of patients with PSC also have inflammatory bowel diseases, usually ulcerative colitis.

• #29 Primary Sclerosing Cholangitis – Liver Foundation

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

  Primary Sclerosing Cholangitis (PSC) is a condition that affects the bile ducts (the tubes that carry bile from the liver to the gallbladder and then to the intestines). […] It’s not clear why some people develop PSC. It’s thought to be triggered by the immune system reacting abnormally to an infection or a toxin. It’s also likely that you inherit the genes for PSC. The cause of PSC is probably different for different people. […] PSC is most common in men aged 30 to 60, and in people with northern European heritage. It seems to be more common in Australia than previously thought. It’s one of the reasons that people need a liver transplant in Australia.

• #30 Primary Sclerosing Cholangitis | CHRISTUS Health

  https://www.christushealth.org/get-care/services-specialties/gastroenterology/liver-pancreas-gallbladder/liver-disease/primary-sclerosing-cholangitis

  Primary sclerosing cholangitis is a rare disorder of the bile ducts in the liver. The exact cause of primary sclerosing cholangitis is not known. It is likely due to a combination of genetic, environmental and immune system factors. […] According to National Association for Rare Disorders, about 60 to 80 percent of people with primary sclerosing cholangitis also have inflammatory bowel disease, or IBD, usually in the form of ulcerative colitis. People with primary sclerosing cholangitis are also more likely to have other autoimmune disorders such as celiac disease, thyroid disease or type 1 diabetes. The reasons these conditions are linked are not clear.

• #31 Primary Sclerosing Cholangitis (PSC) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/primary-sclerosing-cholangitis

  What causes primary sclerosing cholangitis? Researchers have not determined what causes PSC. It may be an autoimmune disease a disorder that causes the bodys immune system to attack healthy tissues though that has not yet been proven. […] The following factors may increase a childs risk for PSC: Family history, Male gender, Autoimmune disease. […] Many people with PSC also have ulcerative colitis. PSC raises the risk for cancer of the gallbladder, bile duct, or colon.

• #32 Primary sclerosing cholangitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-1-41

  There is a well established increased risk of ulcerative colitis in non-smokers when compared to controls. PSC also seems to be a disease of non-smokers, independently of whether the patient has IBD. […] Since the aetiology and pathogenesis of PSC are still unknown, establishing treatment strategies is difficult.

• #33 :: KJR :: Korean Journal of Radiology

  https://www.kjronline.org/DOIx.php?id=10.3348/kjr.2016.17.1.25

  Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. […] Sclerosing cholangitis can be divided into primary sclerosing cholangitis (PSC) of unidentified etiology and secondary sclerosing cholangitis caused by various identifiable etiologies, including immunoglobulin G4-related sclerosing cholangitis (IgG4-SC), recurrent pyogenic cholangitis (RPC), ischemic cholangitis, acquired immunodeficiency syndrome (AIDS)-related cholangitis, and eosinophilic cholangitis. […] Although the pathogenesis remains to be elucidated, many experts accept an autoimmune component.

• #34 Pathology Pearls Post 8: Primary Sclerosing Cholangitis (PSC) | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/pathology-pearls/pathology-pearls-post-8-primary-sclerosing

  Given the absence of etiology for secondary cholangitis such as infection and neoplasia, the histologic findings are suggestive of primary sclerosing cholangitis (PSC). […] Primary biliary cholangitis (PBC) should also be considered in the differential diagnosis of PSC. […] Although less common, IgG4-associated cholangitis can mimic PSC.

• #35 Sclerosing Cholangitis – Types, Causes, Symptoms, Diagnosis, Treatment & Prognosis

  https://www.medindia.net/health/conditions/sclerosing-cholangitis.htm

  Sclerosing cholangitis (SC) is a chronic and progressive cholestatic liver disorder with an unknown aetiology. […] The cause of PSC is not known (idiopathic). It could be due to a combination of genetic and environmental factors. […] In addition, bile duct stones, surgery, parasites or drugs are responsible for some cases of sclerosing cholangitis. […] The cause of Primary Sclerosing Cholangitis (PSC) is primarily unknown. […] Autoimmune diseases. Patient who have PSC show the presence of certain antibodies in the blood although the disease is not an auto-immune one. […] Other causes that are yet to be proved are: Certain types of Gut bacteria. […] A process called „cellular senescence” that is both tumor suppressive and pro-carcinogenic that is involved in how PSC occurs. […] Conditions that cause secondary sclerotic cholangitis include stones in the bile ducts (Choledocolithiasis), cancer of the bile ducts inside and outside the liver, gall bladder cancer liver and prostate gland cancers, surgical trauma causing damage to the bile ducts, ischemic causes like damage to the blood supply of the bile ducts, infectious causes like presence of microorganisms in the bile ducts, disorders of the bile ducts present from birth, toxic causes like chemical agents or drugs, infections of the liver and gallbladder, immune-induced inflammation (cholangitis) or disease (cholangiopathy) of the bile duct, pancreatic disorders like autoimmune and chronic inflammation of pancreas, intra-arterial chemotherapy where the drugs are administered directly to the tumor, chronic obstruction of the bile ducts causing thickening of its walls resulting in recurrent inflammation, sclerosing cholangitis in critically ill patients.

• #36 Sclerosing Cholangitis | Houston, Texas USA |

  https://www.texasliver.com/sclerosing-cholangitis/

  Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by inflammation, destruction and fibrosis of the intrahepatic and extrahepatic bile ducts that leads to cirrhosis of the liver. […] The cause of PSC is unknown but many investigators suspect that it is an autoimmune disease. Other etiologies, such as infectious agents, toxins or recurrent infections of the bile ducts are also possible causes. […] Secondary causes of sclerosing cholangitis must be ruled out when making the diagnosis of PSC. Causes of secondary sclerosing cholangitis include drugs, bile duct cancers, past biliary tree surgery and opportunistic infections of the bile ducts that can cause a similar picture in patients with AIDS. These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests.

• #37 Understanding primary sclerosing cholangitis symptoms

  https://www.antidote.me/blog/primary-sclerosing-cholangitis-symptoms

  The underlying cause of primary sclerosing cholangitis remains uncertain, and there is currently no established treatment for the condition. […] Currently, there is no specific treatment to address the underlying cause of primary sclerosing cholangitis, but research is underway to learn more about the condition and develop potential new therapies for the patient population.

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