Materiały źródłowe

• #1 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Primary sclerosing cholangitis is a progressive disease that damages your bile ducts and your liver. It causes chronic inflammation and scarring. Scar tissue eventually restricts the flow of bile through the ducts and stops the liver from functioning properly. […] Yes. While you might not have symptoms at first, PSC is a progressive disease that gets worse over time. When bile stalls in your bile ducts (cholestasis), it can begin to leak bile toxins into your bloodstream, which makes you sick. It also does progressive damage to your liver. Within 10 to 15 years, this can lead to liver failure. You cant live without a liver. While there are temporizing treatments, there is currently no cure for PSC, except a liver transplant. […] The signs and symptoms of PSC change as the disease becomes more advanced. Up to 50% of people may have no symptoms at all at the time of diagnosis. PSC is often found by accident when testing for other conditions. The first symptoms to develop tend to be vague. They may include: Fatigue, Upper right quadrant abdominal pain, Itchy skin (pruritus). Later-stage symptoms may include: Swollen abdomen, Enlarged liver, Enlarged spleen, Jaundice, Fever, Unintended weight loss.

• #1 Primary sclerosing cholangitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/primary-sclerosing-cholangitis/symptoms-causes/syc-20355797

  Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities. […] Early signs and symptoms often include: Fatigue, Itching, Yellow eyes and skin (jaundice), Abdominal pain. […] Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years. But there’s no reliable way to predict how quickly or slowly the disease will progress for any individual. […] Signs and symptoms that may appear as the disease progresses include: Fever, Chills, Night sweats, Enlarged liver, Enlarged spleen, Weight loss. […] In most people with primary sclerosing cholangitis, the disease progresses slowly. It can eventually lead to liver failure, repeated infections, and tumors of the bile duct or liver.

• #1 Primary Sclerosing Cholangitis (PSC) – British Liver Trust

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

  Symptoms such as itching can be treated with agents such as cholestyramine, rifampicin or naltrexone. […] People with PSC may not have any symptoms associated with fat malabsorption, while others develop symptoms of fat intolerance such as nausea, bloating and abdominal discomfort if they eat even moderate amounts of dietary fat. […] Tiredness is the commonest symptom of PSC. Some people may need to consider making changes to their lifestyle and pacing their daily activities helps to preserve stamina and energy.

• #1 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. In PSC, bile cannot flow out of the liver properly due to narrowing’s (strictures) in the bile ducts. This can lead to a variety of complications, including bile duct infections, cirrhosis, bile duct cancer and/or liver failure although some patients have only mild symptoms. […] PSC is highly variable between people and symptoms can come and go over time. For this reason, your medical history is important. This will establish whether your current symptoms are part of the regular pattern of your disease or if they represent a sudden deterioration. […] Other people may have the following symptoms: Feeling tired (fatigue), Having difficulties concentrating (‘brain fog’), Fever, Feeling generally unwell, Itching (particularly over the palms of the hands and/or soles of the feet), Yellowing of the skin or whites of the eyes (jaundice).

• #1 Primary sclerosing cholangitis – Wikipedia

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

  Typically no symptoms; abdominal pain, fatigue, fever, jaundice […] Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as jaundice, itching, and abdominal pain. […] Abdominal pain affects about 20% of people with PSC. […] Multiple episodes of life-threatening acute cholangitis (infection within the bile ducts) can be seen due to impaired drainage of the bile ducts, which increases the risk of infection. […] The bile duct scarring that occurs in PSC narrows the ducts of the biliary tree and impedes the flow of bile to the duodenum. Eventually, it can lead to cirrhosis of the liver and liver failure. […] PSC progresses slowly and is often asymptomatic, so it can be present for years before it is diagnosed and before it causes clinically significant consequences.

• #1 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) […] PSC often gets worse over time […] Eventually, it may lead to liver failure, infections or tumours in the bile duct or liver. […] The disease gets worse very slowly. Some people have PSC for years before they notice symptoms. Once they are diagnosed, it can take 10 to 15 years or more before liver failure develops. […] PSC often doesn’t cause any symptoms. People only find out they have PSC because they have a blood test for something else. […] If you do have symptoms, at first they might include: Feeling very tired (fatigue), Itching, Yellow eyes and skin (jaundice). […] As the disease gets worse, other symptoms may appear such as: Belly pain, Fever and chills and night sweats (from infections), Weight loss, Dark urine (wee), Light coloured poos, Nausea, Discomfort in the upper abdomen from an enlarged liver or spleen. […] Symptoms may come and go, or they may get worse over time.

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

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

  Symptoms of primary sclerosing cholangitis (PSC) may include pain in the abdomen, or belly. […] Symptoms of PSC include itchy skin. […] Symptoms of a bile duct infection include chills, fever, new or worsening jaundice, pain in the upper right side of the abdomen. […] As the disease gets worse, you may develop cirrhosis. Symptoms of cirrhosis may include swelling of the abdomen from a buildup of fluid, called ascites, confusion or difficulty thinking caused by a buildup of toxins in the brain, called hepatic encephalopathy, gastrointestinal bleeding caused when enlarged veins called varices burst in the esophagus, stomach, or intestines, other symptoms of cirrhosis, such as edema, jaundice, and weight loss. […] Because PSC gets worse slowly, you can have the disease for years before you have any symptoms. Many people have no symptoms when they are first diagnosed with PSC.

• #1 Primary sclerosing cholangitis: MedlinePlus GeneticsLock

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

  Primary sclerosing cholangitis is usually diagnosed around age 40, and for unknown reasons, it affects men twice as often as women. Many people have no signs or symptoms of the condition when they are diagnosed, but routine blood tests reveal liver problems. When apparent, the earliest signs and symptoms of primary sclerosing cholangitis include extreme tiredness (fatigue), discomfort in the abdomen, and severe itchiness (pruritus). […] As the condition worsens, affected individuals may develop yellowing of the skin and whites of the eyes (jaundice) and an enlarged spleen (splenomegaly). Eventually, the buildup of bile damages the liver cells, causing chronic liver disease (cirrhosis) and liver failure. Without bile available to digest them, fats pass through the body. As a result, weight loss and shortages of vitamins that are absorbed with and stored in fats (fat-soluble vitamins) can occur. A fat-soluble vitamin called vitamin D helps absorb calcium and helps bones harden, and lack of this vitamin can cause thinning of the bones (osteoporosis) in people with primary sclerosing cholangitis.

• #1 Primary Sclerosing Cholangitis (PSC): Symptoms & More

  https://www.health.com/primary-sclerosing-cholangitis-8652183

  Primary sclerosing cholangitis (PSC) is a progressive scarring of the liver’s bile ducts. The symptoms of PSC depend on how advanced this progressive condition is. Over time, PSC causes increasing tissue damage and can lead to liver failure, among other issues. The symptoms of PSC depend on how far the condition has advanced. The condition may not initially cause symptoms. Upon diagnosis, about half of people do not yet have any signs. As PSC progresses, the symptoms worsen and evolve, especially if there’s a bile duct infection. The initial symptoms of PSC include those in the abdomen and throughout the body. You might notice the following: Abdominal pain, Pruritis (itchiness), Diarrhea, or loose and watery stools, Jaundice, a yellowing of the skin and whites of the eyes, Fatigue or muscular weakness, Fever. As PSC progresses, it can cause infection and inflammation of the bile ducts. This interrupts the flow of bile, causing symptoms like a new or worsening case of jaundice, chills, and sharp pains in the upper-right quadrant (quarter) of the abdomen. When PSC gets severe, it causes cirrhosis (liver scarring). Symptoms at this stage vary and include: Ascites, or swelling in the abdomen, Hepatic encephalopathy, which causes confusion or difficulty concentrating or thinking, Bleeding in the intestinal tract, stomach, or esophagus (tube connecting the throat to the stomach), Edema, or swelling in the limbs, Jaundice, Weight loss. PSC is a chronic condition that causes slow, progressive damage to the liver. Patients survive an average of 10-20 years after diagnosis. That said, small duct PSC does not always lead to cancer or liver failure and has a better prognosis.

• #1 Primary Sclerosing Cholangitis (PSC) – Jackson Siegelbaum Gastroenterology

  https://www.gicare.com/conditions/primary-sclerosing-cholangitis-psc/

  PSC usually progresses very slowly. Early on there may be no symptoms. Usually the only findings are abnormal laboratory test results. For example, a liver enzyme called alkaline phosphatase may be above normal ranges in the blood. When symptoms do develop, they may be intermittent or persistent. Gradually, they may worsen. The symptoms are caused by two things: the bile is not being drained properly through the bile ducts, and the liver is not doing its job. Bile ducts can become infected, causing chills, fever and upper abdominal tenderness. Itching may occur when bile seeps into the bloodstream. As the disease progresses, chronic fatigue, loss of appetite, weight loss and jaundice (yellowing of skin and eyes) may occur. Finally, in the advanced stages of cirrhosis, extensive swelling can occur in the abdomen and feet. Liver failure may take many years to develop.

• #1 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. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gallbladder and the small intestine, where it helps digest fats and fatty vitamins. […] In patients with PSC, the bile ducts become blocked due to inflammation and scarring or fibrosis. This causes bile to accumulate in the liver, where it gradually damages liver cells and causes cirrhosis, or fibrosis of the liver. As cirrhosis progresses and the amount of scar tissue in the liver increases, the liver slowly loses its ability to function. The scar tissue may block drainage of the bile ducts leading to infection of the bile. […] PSC advances very slowly. Many patients may have the disease for years before symptoms develop. Symptoms may remain at a stable level, they may come and go, or they may progress gradually. Liver failure may occur 10-15 years after diagnosis, but this may take even longer for some PSC patients. Many people with PSC will ultimately need a liver transplant, typically about 10 years after being diagnosed with the disease. PSC may also lead to bile duct cancer. Endoscopy and MRI tests may be done to monitor the disease.

• #1 Mayo Clinic Q and A: Primary sclerosing cholangitis – Mayo Clinic News Network

  https://newsnetwork.mayoclinic.org/discussion/mayo-clinic-q-and-a-primary-sclerosing-cholangitis/

  Primary sclerosing cholangitis, or PSC, is a chronic inflammatory disease of the bile ducts that may lead to severe liver damage and liver failure. […] Signs and symptoms of advanced PSC include yellowing of the eyes or skin (jaundice), itching, abdominal pain, leg swelling and infection. […] Some people with PSC progress and need a transplant soon after diagnosis. However, many carry the disease for decades without it showing any significant progression or symptoms.

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

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

  PSC is a progressive disease, with evolution to biliary cirrhosis and malignancy in the majority of patients (65). […] In a large population-based study, in which 92% of patients were treated with UDCA, the estimated survival time from diagnosis until PSC-related death or LT was 21 years, compared to median 13 years transplant-free survival in a cohort from three liver transplant centers (37). […] Though patients who are asymptomatic have a better prognosis than those with symptoms at diagnosis, symptoms often develop over time (3). […] However, there is significant variation among individuals and between different subtypes. […] For example, patients with small duct PSC disease generally have better outcomes than those with classic disease and do not seem to develop CCA, unless the disease has progressed to large-duct PSC (21).

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

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

  Other favorable prognostic factors include younger age at diagnosis, and female sex (36). […] On the other hand, poor prognostic factors include extensive intrahepatic or extrahepatic biliary strictures (66), DSs (67), recurrent cholangitis (68), UC [compared to Crohns disease (CD) or no IBD] (36), evidence of liver synthetic dysfunction and cirrhosis with portal hypertension.

• #1 Primary Sclerosing Cholangitis (Causes, Symptoms and Treatment)

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

  Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterised by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts, resulting in multifocal biliary strictures. […] PSC commonly affects males with a median age at diagnosis of 35 years. […] May be asymptomatic (presenting with abnormal LFTs or hepatomegaly). […] Jaundice and pruritus. […] Right upper quadrant abdominal pain. […] Fatigue, weight loss, fevers and sweats. […] May present with complications (see 'Complications and their management’, below). […] Later stages: signs of cirrhosis, portal hypertension or hepatic failure. […] PSC is a progressive disease of the liver characterised by inflammation and destruction of the intrahepatic and/or extrahepatic bile ducts, leading to fibrosis and ultimately liver failure, cirrhosis and an increased risk of malignancy. […] PSC can be classified into small-duct or large-duct types, which seems to affect prognosis: Small-duct PSC has a better prognosis, with longer transplant-free survival. […] Prognosis after liver transplant: Survival post-transplant is about 94% 1 year, 86% at 5 years and 70% at 10 years.

• #1 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Primary sclerosing cholangitis is a slow progressing disease. But as your bile ducts and liver become increasingly scarred, their functions will increasingly fail. This can lead to: Metabolic diseases, Portal hypertension, Infections, Cancer. […] Currently, there is no treatment that can slow or stop primary sclerosing cholangitis from progressing. You can treat some of the symptoms and complications directly. […] After diagnosis, the average life expectancy ranges between 10 and 20 years. A liver transplant can give you a new lease on life. However, in 15% to 20% of cases, PSC may return after a liver transplant. When this happens, the new liver may fail. The average life expectancy in this case is about nine months.

• #1 Primary Sclerosing Cholangitis | Children’s Liver Disease Foundation

  https://childliverdisease.org/liver-information/childhood-liver-conditions/primary-sclerosing-cholangitis/

  If the disease progresses, the following complications may be experienced: Biliary strictures narrowing of a bile duct from scar tissue within the duct itself. A stricture can block the release of bile and lead to infection (cholangitis). An ERCP may be required. Progressive itch, Portal hypertension high blood pressure in the portal vein which may occur due to scarring of the liver or a blockage in the portal vein. The spleen may become enlarged (splenomegaly) due to a back flow of blood from the liver and cause abdominal distension. Varices swollen veins in the intestine, oesophagus and stomach which can bleed if not treated. An endoscopy is usually required. Ascites collecting of fluid in the abdomen. Ascites can be managed by diuretic medicines to increase urine output and minimise fluid accumulation. […] Development of bile duct cancer (cholangiocarcinoma). This is extremely rare in children.

• #1 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

  Onset is usually insidious, with progressive fatigue and then pruritus. Up to 40% of patients can present with symptoms including abdominal pain, pruritis, diarrhea, jaundice, fatigue, and fevers. Steatorrhea and deficiencies of fat-soluble vitamins can develop. Persistent jaundice harbingers advanced disease. Symptomatic gallstones and choledocholithiasis tend to develop in about 75% of patients. […] Some patients, asymptomatic until late in the course, first present with hepatosplenomegaly or cirrhosis. Primary sclerosing cholangitis (PSC) tends to slowly and inexorably progress. The terminal phase involves decompensated cirrhosis, portal hypertension, ascites, and liver failure. […] Cholangiocarcinoma develops in 10 to 15% of patients.

• #1 Primary Sclerosing Cholangitis | Diagnosis and Treatment

  https://patient.info/digestive-health/primary-sclerosing-cholangitis

  The scarring causes narrowing of these bile ducts which results in bile building up in the liver. The bile can then damage the liver cells. Eventually, the scar tissue can spread throughout the liver, causing cirrhosis and liver failure. Cirrhosis is a serious condition where normal liver tissue is replaced by scar tissue (fibrosis). It tends to progress slowly and often does not cause symptoms in its early stages. However, as the function of the liver gradually becomes worse, serious problems can develop. […] The course of PSC is variable. The outlook and life expectancy are poorer in older patients, those who have an enlarged liver and spleen, and in patients who are persistently jaundiced. There is also an increased risk of developing cancer of the bile duct (cholangiocarcinoma) or bowel (colorectal) cancer. The risk of developing bowel cancer is higher in people with PSC who also have ulcerative colitis.

• #1 Primary Sclerosing Cholangitis | Liver Canada

  https://liver.ca/primary-biliary-cholangitis/

  Primary sclerosing cholangitis is unpredictable and not well understood. A person can have the disease for many years before symptoms develop. When a patient does experience symptoms they may include: […] If a person who suffers from primary sclerosing cholangitis develops fever and chills, they should go to the hospital immediately. Eventually, in the advanced stages of PSC, cirrhosis may develop with signs of liver failure. […] The impact of primary sclerosing cholangitis on a patient can be unpredictable. Many people are able to live a normal life with PSC while others progress to advanced liver damage and eventually liver failure. If symptoms begin to develop, common symptoms like fatigue, pain, and severe itching can make it difficult for people with this condition to carry out daily tasks.

• #1 Primary Sclerosing Cholangitis: Symptoms and Treatment Guide

  https://www.medicoverhospitals.in/articles/primary-sclerosing-cholangitis

  PSC often progresses slowly, and symptoms may not be apparent in the early stages. As the disease advances, common symptoms include: Fatigue, Itching, Jaundice (yellowing of the skin and eyes), Abdominal pain, Chills and fever. It’s important to note that these symptoms can overlap with other liver diseases, making diagnosis challenging. […] The progression of PSC varies from person to person. Some individuals may live for many years without significant symptoms, while others may experience rapid disease progression. Regular monitoring and early intervention are crucial to managing the disease effectively. […] It can cause fatigue, itching, abdominal pain, and complications affecting daily activities, requiring lifestyle adjustments and regular medical care.

• #1 Primary Sclerosing Cholangitis – PSC | Choose the Right Test

  https://arupconsult.com/content/primary-sclerosing-cholangitis-psc

  An increased serum immunoglobulin G4 (IgG4) may correlate with a more severe disease course. Approximately 10% of patients present with elevated serum IgG4, and these patients tend to have a more aggressive disease course with no treatment. […] Liver biochemistry should be assessed every 3-4 months in patients with PSC with the aim of identifying signs of strictures, tumors, or AIH. […] Patients with PSC have a higher likelihood of developing several different types of cancer, so regular cancer screenings are an important part of managing PSC.

• #1 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. […] Further care is dictated by the stage and degree of progression of the disease. It involves care by a gastroenterologist, who must monitor the patient’s condition with frequent laboratory tests and maintain vigilance for the many complications of PSC. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression. […] Nearly 50% of deaths in patients with primary sclerosing cholangitis (PSC) are due to cancer. All patients with PSC should undergo surveillance for malignancy.

• #1 Primary Sclerosing Cholangitis (PSC) | UPMC | Pittsburgh PA

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

  Even though PSC is a serious disease. Many people who have it don’t have symptoms right away. […] As the disease worsens, symptoms can come and go. […] Symptoms of PSC include: Pain in the upper right part of the belly. Extreme itching. Unexplained weight loss. Yellowed skin or eyes known as jaundice. Fatigue. Chills. Night sweats. […] See your doctor if you have these symptoms. They could have many causes, so expect your doctor to run a range of tests to confirm a diagnosis. […] Doctors may suspect PSC when routine blood tests have abnormal results, even if you don’t have symptoms. […] If you have PSC, your doctor will: Track your liver health closely to find and treat any new problem right away. Treat any infections and manage symptoms like pain or jaundice. […] If PSC causes severe cirrhosis or liver failure, you may need a liver transplant. Transplant provides the only known cure for PSC.

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

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

  Unfortunately, there is no known medical treatment that slows down the rate of liver disease in people with PSC. However it is important to note that the course of PSC is very variable and some patients remain asymptomatic for many years. […] Approximately 3 out of 20 people can also develop a cancer in their bowel or bile ducts. Bile duct cancers are most often diagnosed in the first two years after PSC diagnosis and less commonly picked up later in the disease course.

• #2 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. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gallbladder and the small intestine, where it helps digest fats and fatty vitamins. […] In patients with PSC, the bile ducts become blocked due to inflammation and scarring or fibrosis. This causes bile to accumulate in the liver, where it gradually damages liver cells and causes cirrhosis, or fibrosis of the liver. As cirrhosis progresses and the amount of scar tissue in the liver increases, the liver slowly loses its ability to function. The scar tissue may block drainage of the bile ducts leading to infection of the bile. […] PSC advances very slowly. Many patients may have the disease for years before symptoms develop. Symptoms may remain at a stable level, they may come and go, or they may progress gradually. Liver failure may occur 10-15 years after diagnosis, but this may take even longer for some PSC patients. Many people with PSC will ultimately need a liver transplant, typically about 10 years after being diagnosed with the disease. PSC may also lead to bile duct cancer. Endoscopy and MRI tests may be done to monitor the disease.

• #2 Primary sclerosing cholangitis: MedlinePlus GeneticsLock

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

  Primary sclerosing cholangitis is usually diagnosed around age 40, and for unknown reasons, it affects men twice as often as women. Many people have no signs or symptoms of the condition when they are diagnosed, but routine blood tests reveal liver problems. When apparent, the earliest signs and symptoms of primary sclerosing cholangitis include extreme tiredness (fatigue), discomfort in the abdomen, and severe itchiness (pruritus). […] As the condition worsens, affected individuals may develop yellowing of the skin and whites of the eyes (jaundice) and an enlarged spleen (splenomegaly). Eventually, the buildup of bile damages the liver cells, causing chronic liver disease (cirrhosis) and liver failure. Without bile available to digest them, fats pass through the body. As a result, weight loss and shortages of vitamins that are absorbed with and stored in fats (fat-soluble vitamins) can occur. A fat-soluble vitamin called vitamin D helps absorb calcium and helps bones harden, and lack of this vitamin can cause thinning of the bones (osteoporosis) in people with primary sclerosing cholangitis.

• #2 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) […] PSC often gets worse over time […] Eventually, it may lead to liver failure, infections or tumours in the bile duct or liver. […] The disease gets worse very slowly. Some people have PSC for years before they notice symptoms. Once they are diagnosed, it can take 10 to 15 years or more before liver failure develops. […] PSC often doesn’t cause any symptoms. People only find out they have PSC because they have a blood test for something else. […] If you do have symptoms, at first they might include: Feeling very tired (fatigue), Itching, Yellow eyes and skin (jaundice). […] As the disease gets worse, other symptoms may appear such as: Belly pain, Fever and chills and night sweats (from infections), Weight loss, Dark urine (wee), Light coloured poos, Nausea, Discomfort in the upper abdomen from an enlarged liver or spleen. […] Symptoms may come and go, or they may get worse over time.

• #2 Primary Sclerosing Cholangitis | Children’s Pittsburgh

  https://www.chp.edu/our-services/transplant/liver/education/liver-disease-states/primary-sclerosing-cholangitis

  Many people with primary sclerosing cholangitis symptoms are non-existent when a patient is first diagnosed; others have already developed chronic liver disease. […] The most common nonspecific symptom is fatigue. Other less common symptoms include severe itching, weight loss, jaundice, abdominal pain, and unexplained fever. Primary sclerosing cholangitis often progresses slowly, so a person can have the disease for years before symptoms develop. […] Primary sclerosing cholangitis is a progressive disease often leading to biliary cirrhosis within 10 to 15 years.

• #2 Primary Sclerosing Cholangitis (PSC)

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

  Many people with PSC do not get symptoms, especially in the early stages of the disease. When symptoms do occur the most common are fatigue, pruritus, or itching of the skin, and jaundice, a yellowing of the skin and eyes. These symptoms may come and go, but they may worsen over time. As the disease continues, the bile ducts may become infected, which can lead to episodes of fever, chills and abdominal pain. […] Over time, many PSC patients will continue to suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery.

• #2 Primary Sclerosing Cholangitis (PSC) – British Liver Trust

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

  Primary Sclerosing Cholangitis is an uncommon chronic liver disease in which the bile ducts inside and outside the liver progressively decrease in size due to inflammation and scarring (fibrosis). […] Many people have no symptoms at first and the disease is only discovered because of abnormal results of routine blood tests in patients with ulcerative colitis or Crohns disease. In some people PSC does not produce any symptoms. Most people have few or no symptoms for many years. […] Common early symptoms of PSC are: tiredness, some abdominal discomfort in the right upper abdomen. […] Late symptoms of PSC are: itching, jaundice yellowing of the skin and whites of the eyes, episodes of fever, shaking and chills can be distressing but are uncommon. […] Liver failure may ultimately develop PSC may be occasionally complicated by the development of bile duct cancer.

• #2 Primary Sclerosing Cholangitis Symptoms, Treatment & Prognosis

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

  Primary sclerosing cholangitis describes a disease process in which the bile ducts in the liver become inflamed, narrow and prevent bile from flowing properly. […] As the disease progress, liver cells die and are replaced by scar tissue. This is called cirrhosis and is associated with liver failure. […] In its early stages, PSC is asymptomatic (there are no symptoms). It is only when the person develops cholestasis because of the inability of bile to drain from the liver, do symptoms occur. These early symptoms include itching and fatigue. As the bilirubin levels in the blood become elevated, jaundice or a yellowish tinge to the skin may occur. Since there may be liver inflammation, the person may complain of pain beneath the ribs in the right upper quadrant of the abdomen. […] Because PSC is a progressive disease, symptoms may come and go over several months and years after the diagnosis is made.

• #2 Primary sclerosing cholangitis (PSC) | CUH

  https://www.cuh.nhs.uk/patient-information/primary-sclerosing-cholangitis-psc/

  PSC often causes no symptoms and is identified because of abnormal liver blood tests. The symptoms that PSC can cause are tiredness, itching, yellow skin (jaundice) and fevers. […] Please make contact if you develop any of the following symptoms/problems, as they may indicate a deterioration in your condition: itching, pain, jaundice, dark urine, pale stools, high fever. […] Symptoms include fever, shivers, chills, abdominal pain, nausea, vomiting and jaundice.

• #2 IBD and Primary Sclerosing Cholangitis (PSC)

  https://www.verywellhealth.com/ibd-and-primary-sclerosing-cholangitis-psc-1941767

  Primary sclerosing cholangitis (PSC) is a rare and chronic disease of the liver that causes inflammation and narrowing of the bile ducts inside and outside of the liver. […] PSC does progress slowly in most cases, but it can also be unpredictable and life-threatening. People with PSC can receive treatment to relieve symptoms and help them lead an active life. […] Some people have no symptoms at diagnosis or even for several years after, according to the Mayo Clinic, reporting early signs of PSC include fatigue, itching, jaundice, and abdominal pain. The symptoms worsen as the disorder progresses with the following signs becoming more common. […] Fever […] Chills […] Night sweats […] Enlarged liver […] Enlarged spleen […] Weight loss.

• #2 Primary Sclerosing Cholangitis (PSC) | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/primary-sclerosing-cholangitis-psc.html

  Primary sclerosing cholangitis (PSC) is a rare liver disease that causes the bile ducts inside and outside the liver to become inflamed and scarred. They slowly narrow until bile backs up into your liver and causes liver damage. […] Symptoms of PSC develop slowly. You may have PSC for many years without any symptoms. After symptoms develop, they may come and go. But they often get worse over time. Bile that backs up into your liver may over time cause a type of liver scarring known as cirrhosis. This leads to liver failure. Symptoms of PSC include: Itchy skin. Extreme tiredness (fatigue). Belly pain. Yellowing of the skin and eyes, called jaundice. Chills and fever from infection of your bile ducts. […] PSC progresses slowly. But after about 10 to 15 years, it may lead to liver failure. Many people with liver failure respond well to liver transplant surgery.

• #2 Primary Sclerosing Cholangitis Symptoms, Treatment & Prognosis

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

  As the disease progresses, cirrhosis may occur leading to decreased liver function and its associated signs and symptoms. These include bleeding from the esophagus and stomach due to varices, ascites (fluid collection in the abdomen) because of poor protein production by the liver, easy bruising because of decreased platelets in the blood because they are trapped in the enlarged spleen, and confusion due to hepatic encephalopathy because the liver cannot clear waste products of metabolism. […] PSC is a progressive disease that damages the bile ducts and reduces the bile flow, ultimately leading to portal hypertension, cirrhosis, and liver failure.

• #2 Primary Sclerosing Cholangitis | Children’s Liver Disease Foundation

  https://childliverdisease.org/liver-information/childhood-liver-conditions/primary-sclerosing-cholangitis/

  If the disease progresses, the following complications may be experienced: Biliary strictures narrowing of a bile duct from scar tissue within the duct itself. A stricture can block the release of bile and lead to infection (cholangitis). An ERCP may be required. Progressive itch, Portal hypertension high blood pressure in the portal vein which may occur due to scarring of the liver or a blockage in the portal vein. The spleen may become enlarged (splenomegaly) due to a back flow of blood from the liver and cause abdominal distension. Varices swollen veins in the intestine, oesophagus and stomach which can bleed if not treated. An endoscopy is usually required. Ascites collecting of fluid in the abdomen. Ascites can be managed by diuretic medicines to increase urine output and minimise fluid accumulation. […] Development of bile duct cancer (cholangiocarcinoma). This is extremely rare in children.

• #2 Primary sclerosing cholangitis – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/847?locale=it

  Primary sclerosing cholangitis is a cholestatic liver disease causing continued destruction of the bile ducts, cirrhosis, and end-stage liver disease. […] Patients may be asymptomatic at diagnosis but develop symptoms of pruritus and jaundice. […] Complications include dominant/relevant strictures (focal areas of tight narrowing of the extrahepatic biliary tree that develops in 40% to 50% of patients as a result of progressive structuring), cholangitis, cholangiocarcinoma, and end-stage liver disease (due to chronic progressive biliary fibrosis). […] Other diagnostic factors include abdominal pain, pruritus, fatigue, weight loss, fever, jaundice, steatorrhea, splenomegaly, ascites, and encephalopathy. […] Liver failure and cholangiocarcinoma are the leading causes of death.

• #2 Primary Sclerosing Cholangitis (PSC): Symptoms & More

  https://www.health.com/primary-sclerosing-cholangitis-8652183

  Primary sclerosing cholangitis (PSC) is a progressive scarring of the liver’s bile ducts. The symptoms of PSC depend on how advanced this progressive condition is. Over time, PSC causes increasing tissue damage and can lead to liver failure, among other issues. The symptoms of PSC depend on how far the condition has advanced. The condition may not initially cause symptoms. Upon diagnosis, about half of people do not yet have any signs. As PSC progresses, the symptoms worsen and evolve, especially if there’s a bile duct infection. The initial symptoms of PSC include those in the abdomen and throughout the body. You might notice the following: Abdominal pain, Pruritis (itchiness), Diarrhea, or loose and watery stools, Jaundice, a yellowing of the skin and whites of the eyes, Fatigue or muscular weakness, Fever. As PSC progresses, it can cause infection and inflammation of the bile ducts. This interrupts the flow of bile, causing symptoms like a new or worsening case of jaundice, chills, and sharp pains in the upper-right quadrant (quarter) of the abdomen. When PSC gets severe, it causes cirrhosis (liver scarring). Symptoms at this stage vary and include: Ascites, or swelling in the abdomen, Hepatic encephalopathy, which causes confusion or difficulty concentrating or thinking, Bleeding in the intestinal tract, stomach, or esophagus (tube connecting the throat to the stomach), Edema, or swelling in the limbs, Jaundice, Weight loss. PSC is a chronic condition that causes slow, progressive damage to the liver. Patients survive an average of 10-20 years after diagnosis. That said, small duct PSC does not always lead to cancer or liver failure and has a better prognosis.

• #2 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

  Onset is usually insidious, with progressive fatigue and then pruritus. Up to 40% of patients can present with symptoms including abdominal pain, pruritis, diarrhea, jaundice, fatigue, and fevers. Steatorrhea and deficiencies of fat-soluble vitamins can develop. Persistent jaundice harbingers advanced disease. Symptomatic gallstones and choledocholithiasis tend to develop in about 75% of patients. […] Some patients, asymptomatic until late in the course, first present with hepatosplenomegaly or cirrhosis. Primary sclerosing cholangitis (PSC) tends to slowly and inexorably progress. The terminal phase involves decompensated cirrhosis, portal hypertension, ascites, and liver failure. […] Cholangiocarcinoma develops in 10 to 15% of patients.

• #2 Quick Facts:Primary Sclerosing Cholangitis – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/quick-facts-liver-and-gallbladder-disorders/gallbladder-and-bile-duct-disorders/primary-sclerosing-cholangitis

  Primary sclerosing cholangitis is a long-term disease that causes swelling, scarring, and narrowing of the bile ducts. […] The first symptoms are weakness, tiredness, and itchy skin. […] Symptoms of primary sclerosing cholangitis include: Feeling weak and tired, Itchy skin, Yellow eyes and skin (jaundice), Sometimes, pain in your upper belly. […] Later you may have: Osteoporosis (weak bones), Bruising and bleeding easily, Greasy and bad-smelling stool, Gallstones, Liver problems, such as cirrhosis. […] Liver failure usually happens about 12 years after diagnosis.

• #2 Primary Sclerosing Cholangitis: Symptoms, Treatment & Diagnosis

  https://my.clevelandclinic.org/health/diseases/23569-primary-sclerosing-cholangitis

  Primary sclerosing cholangitis is a slow progressing disease. But as your bile ducts and liver become increasingly scarred, their functions will increasingly fail. This can lead to: Metabolic diseases, Portal hypertension, Infections, Cancer. […] Currently, there is no treatment that can slow or stop primary sclerosing cholangitis from progressing. You can treat some of the symptoms and complications directly. […] After diagnosis, the average life expectancy ranges between 10 and 20 years. A liver transplant can give you a new lease on life. However, in 15% to 20% of cases, PSC may return after a liver transplant. When this happens, the new liver may fail. The average life expectancy in this case is about nine months.

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

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

  Other favorable prognostic factors include younger age at diagnosis, and female sex (36). […] On the other hand, poor prognostic factors include extensive intrahepatic or extrahepatic biliary strictures (66), DSs (67), recurrent cholangitis (68), UC [compared to Crohns disease (CD) or no IBD] (36), evidence of liver synthetic dysfunction and cirrhosis with portal hypertension.

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

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

  PSC is a progressive disease, with evolution to biliary cirrhosis and malignancy in the majority of patients (65). […] In a large population-based study, in which 92% of patients were treated with UDCA, the estimated survival time from diagnosis until PSC-related death or LT was 21 years, compared to median 13 years transplant-free survival in a cohort from three liver transplant centers (37). […] Though patients who are asymptomatic have a better prognosis than those with symptoms at diagnosis, symptoms often develop over time (3). […] However, there is significant variation among individuals and between different subtypes. […] For example, patients with small duct PSC disease generally have better outcomes than those with classic disease and do not seem to develop CCA, unless the disease has progressed to large-duct PSC (21).

• #2 PSC — Primary Sclerosing Cholangitis – Center For Digestive Health

  https://troygastro.com/conditions/psc-primary-sclerosing-cholangitis/

  Primary sclerosing cholangitis is a health condition that affects the bile ducts, which are responsible for transporting bile from the liver to the small intestine. Bile is a fluid involved in digestion, and when a person develops primary sclerosing cholangitis, they have inflammation that leads to scarring in the bile ducts. […] Over time, scars in the bile ducts cause them to become hard and narrow, slowly leading to liver damage as the disease progresses. Disease progression is typically slow with primary sclerosing cholangitis, so people who have the condition may generally feel pretty well in the early stages. That being said, disease progression can vary between people. […] Primary sclerosing cholangitis can lead to serious complications over time. For instance, inflammation and scarring from the condition can lead to liver cirrhosis, death of liver cells, and eventually liver failure. Additional complications include repeated bile duct infections as well as high blood pressure in an important blood vessel called the portal vein. The latter can cause veins to become swollen and weak, ultimately leading to life-threatening bleeding. Other complications include thinning bones and increased risk of colon cancer and bile duct cancer.

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

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

  Unfortunately, there is no known medical treatment that slows down the rate of liver disease in people with PSC. However it is important to note that the course of PSC is very variable and some patients remain asymptomatic for many years. […] Approximately 3 out of 20 people can also develop a cancer in their bowel or bile ducts. Bile duct cancers are most often diagnosed in the first two years after PSC diagnosis and less commonly picked up later in the disease course.

• #2 Primary Sclerosing Cholangitis (PSC) – British Liver Trust

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

  Symptoms such as itching can be treated with agents such as cholestyramine, rifampicin or naltrexone. […] People with PSC may not have any symptoms associated with fat malabsorption, while others develop symptoms of fat intolerance such as nausea, bloating and abdominal discomfort if they eat even moderate amounts of dietary fat. […] Tiredness is the commonest symptom of PSC. Some people may need to consider making changes to their lifestyle and pacing their daily activities helps to preserve stamina and energy.

• #2 Primary Sclerosing Cholangitis – PSC | Choose the Right Test

  https://arupconsult.com/content/primary-sclerosing-cholangitis-psc

  Primary sclerosing cholangitis (PSC) is a progressive autoimmune liver disease (ALD) that often leads to cirrhosis and may eventually require liver transplantation. […] Symptoms, when present, include pruritus, fatigue, jaundice, and gastrointestinal bleeding. […] Approximately 80% of patients with primary sclerosing cholangitis (PSC) also have inflammatory bowel disease (IBD), most commonly ulcerative colitis (UC). […] The presence and severity of IBD does not appear to be associated with long-term outcomes of PSC, including the need for liver transplantation and likelihood of PSC recurrence after transplantation. […] Elevated serum bilirubin is a marker of poor prognosis. An increased AST:ALT ratio may be an indicator of cirrhosis and poor prognosis; other indications of cirrhosis include elevated prothrombin time, low albumin, and low platelets.

• #2 Cholangitis: Symptoms, Causes, Treatment, and More

  https://www.healthline.com/health/cholangitis

  Chronic cholangitis doesn’t go away, but treatment is still necessary to help improve symptoms, prevent complications, and protect your health. […] Life expectancy for people with the condition varies based on factors like which type you have, how long you’ve had it, and how quickly you started treatment. Early treatment can help improve outcomes, prevent symptoms, and avoid serious health complications.

• #2 Primary Sclerosing Cholangitis Treatment & Management: Approach Considerations, Liver Transplantation, Diet and Activity

  https://emedicine.medscape.com/article/187724-treatment

  No approved or proven therapy exists for primary sclerosing cholangitis (PSC). Pharmacotherapy is aimed at treating symptoms and managing complications. […] Further care is dictated by the stage and degree of progression of the disease. It involves care by a gastroenterologist, who must monitor the patient’s condition with frequent laboratory tests and maintain vigilance for the many complications of PSC. […] Liver transplantation is the only therapy that can alter the eventual outcome, with reported posttransplant survival rates of 85% at 5 years and 70% at 10 years. […] Endoscopic or percutaneous transhepatic dilation of dominant strictures, with or without stenting, has been shown to alleviate cholestasis and to improve laboratory test results; however, it is not currently believed to affect disease progression. […] Nearly 50% of deaths in patients with primary sclerosing cholangitis (PSC) are due to cancer. All patients with PSC should undergo surveillance for malignancy.

• #3 Primary sclerosing cholangitis: Symptoms, causes, diagnosis

  https://www.medicalnewstoday.com/articles/190952

  Tiredness is a symptom of primary sclerosing cholangitis. […] Most people with early-stage PSC have no symptoms. A doctor may only detect the disease during a routine test. A blood test can indicate high levels of liver enzymes, particularly alkaline phosphatase. […] Symptoms occur for the following reasons: Bile is not draining properly through the ducts. The liver is damaged. The liver is starting to fail. […] If the bile ducts become infected, it can cause chills, fever, and upper abdominal tenderness. […] Itching may occur when bile seeps into the bloodstream. […] As the disease progresses, a person may develop: persistent tiredness or fatigue, a loss of appetite, weight loss, jaundice. […] A person in the advanced stages of cirrhosis may experience extensive swelling in the abdomen and feet. […] Liver failure may occur 10-15 years after a person receives a PSC diagnosis, or it may take longer, depending on the individual.

• #4 Primary Sclerosing Cholangitis Treatment, Diagnosis & Symptoms

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

  Most individuals with early primary sclerosing cholangitis have no symptoms, and the presence of primary sclerosing cholangitis is recognized only because of abnormally elevated blood levels of liver enzymes (particularly alkaline phosphatase levels) that often are performed along with a routine physical examination. […] Early symptoms of primary sclerosing cholangitis include fatigue and bodily itching (pruritus). As the disease progresses, individuals may develop jaundice (yellowing of skin and darkening of urine). Jaundice is due to the accumulation of bilirubin in the body. The bilirubin accumulates because it is not able to be eliminated in the bile due to extensive obstruction of the bile ducts. The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow. The reason for the pruritus is not entirely known. It may be due to accumulation of bile salts in the body, also as a result of obstruction of the bile ducts.

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