Primary sclerosing cholangitis refers to chronic inflammation of the bile ducts. It causes scarring hardening, resulting in narrowing of the bile ducts.
What is primary sclerosing cholangitis?
Primary sclerosing cholangitis (PSC) is a specific type of cholangitis (bile duct inflammation). It is classified as an autoimmune disease. As part of the disease, affected individuals suffer from progressive and chronic bile duct inflammation that occurs both inside and outside the liver. Primary sclerosing cholangitis is closely associated with other autoimmune diseases such as ulcerative colitis, a chronic inflammatory bowel disease. As the disease progresses, the autoimmune disease leads to scarring of the bile ducts. This in turn results in bile stasis. In the worst case, cirrhosis of the liver may develop, requiring liver transplantation for treatment. Primary sclerosing cholangitis is relatively rare. It is estimated to affect 1 to 5 out of every 100,000 people. It occurs two or three times as often in men as in women. People between the ages of 30 and 50 are particularly affected. In 70 out of 100 patients, ulcerative colitis is also present, and in some cases Crohn’s disease. This disease is also a chronic inflammatory bowel disease. Furthermore, other autoimmune diseases such as Sjögren’s syndrome or autoimmune hepatitis may present simultaneously with primary sclerosing cholangitis.
Causes
What causes primary sclerosing cholangitis is still unclear. In some families, it shows up clustered among first-degree relatives. Therefore, physicians consider a genetic influence to be certain. Together with other factors, this causes the onset of the disease. At the same time, an immunological genesis is assumed, as in the case of an autoimmune disease. Thus, faulty immune reactions at the bile duct mucosa lead to the development of primary sclerosing cholangitis. However, microorganisms in the digestive region such as bacteria are also thought to play a role.
Symptoms, complaints, and signs
In the early stages of primary sclerosing cholangitis, there are no symptoms yet, so the disease is usually not noticed for a long time. Complaints do not appear until the damaged bile ducts restrict the functions of the liver. In this case, the affected person suffers from excruciating itching, weight loss, fatigue, and a yellowish discoloration of the skin and mucous membrane, which doctors also refer to as jaundice. In addition, the patient is sensitive to pressure and suffers from pain in the right upper abdomen. Most patients also have the chronic inflammatory bowel disease ulcerative colitis. Furthermore, there is an increased risk of bacterial inflammation occurring in the bile ducts. Such bacterial cholangitis presents in episodes and triggers abdominal pain on the right side of the abdomen, weakness, and fever.
Diagnosis and course of the disease
If primary sclerosing cholangitis is suspected, the patient should consult a gastroenterologist or an internist who specializes in the treatment of such diseases. The first step in the examination is to take the patient’s medical history. The physician asks the patient whether he or she suffers from cramping or constant abdominal pain, whether he or she has ulcerative colitis, whether he or she has had gallstones in the past, or whether he or she has a fever. The anamnesis is followed by a physical examination, during which the physician checks the patient’s skin. For example, a yellowish discoloration of the skin is considered an indication of liver damage. Using a stethoscope, the doctor listens to the bowel sounds. In this way, he checks the stool and air content of the intestines. However, primary sclerosing cholangitis cannot always be detected by physical examination. For this reason, further examinations take place. These include a blood test to determine liver values, which is used to detect cholestasis parameters such as alkaline phosphatase or gamma-GT.Sonography (ultrasound examination) can be performed to observe the bile ducts and liver, which can be used to differentiate primary sclerosing cholangitis from other diseases. If the suspicion of PSC is confirmed, a more detailed examination of the bile ducts must be performed, which is done with magnetic resonance cholangiopancreaticography (MRCP) and endoscopic retrograde cholangiopancreaticography (ERCP). In this context, ERCP can also be used therapeutically. The course of primary sclerosing cholangitis varies from individual to individual. If the chronic inflammation progresses, there is a risk of secondary damage such as fibrosis, in which the connective tissue increases pathologically, and liver cirrhosis, which leads to a loss of liver function. In addition, there is an increased risk of cancer.
Complications
Primary sclerosing cholangitis (PSC) can be individualized and exceedingly variable. Therefore, in some cases, there is a threat of serious complications. However, these do not affect every individual patient. A common sequela of primary sclerosing cholangitis is bile duct stenosis. Due to the narrowing, there is an increased risk of gallstones forming, which are located above the constrictions. Furthermore, a bacterial bile duct infection (acute cholangitis) may occur, which is manifested by symptoms such as colicky pain, fever and chills. Among the serious complications of PSC is cirrhosis of the liver. It is caused by a chronic buildup of bile in the liver. Physicians also speak of secondary biliary cirrhosis. This involves a nodular remodeling of the liver, which shrinks and increasingly loses its functions. It is not uncommon for such cases to require liver transplantation. Primary sclerosing cholangitis also increases the risk of developing cancer. Thus, 13 to 14 percent of all patients are at risk of developing hepatobiliary carcinoma (cholangiocarcinoma). At the same time, the risk of colon cancer, hepatocellular carcinoma, pancreatic cancer and gallbladder cancer increases. For this reason, patients must undergo regular cancer screening. Other possible sequelae of primary sclerosing cholangitis include rheumatic complaints, osteoporosis (bone loss), and deficiencies of vitamin A, vitamin D, vitamin E, and vitamin K. These vitamins have in common with each other that they have fat-soluble properties.
When should you go to the doctor?
Symptoms such as jaundice, fatigue, and weight loss indicate primary sclerosing cholangitis. A visit to the doctor is indicated if these and other symptoms typical of the disease occur. If abdominal pain or the itching that often occurs in the area of the arms and legs are also present, a doctor must be consulted on the same day. People suffering from diseases of the bile ducts are particularly at risk and should contact their family doctor if the described signs of the disease occur. People with chronic gastrointestinal complaints are also among the risk groups. Anyone with cases of primary sclerosing cholangitis in the family should consult a physician. In addition to the family doctor, the chronic condition can be taken to an internist or a specialist in diseases of the gallbladder. The condition is usually treated as an inpatient in a specialized clinic. In case of chronic disease, the patient must keep close consultation with the doctor. The medical professional should be informed of any unusual symptoms so that adjustments in treatment can be made. If cirrhosis of the liver occurs, the emergency physician must be called and first aid given if necessary. Any subsequent symptoms, such as bone loss or tumors of the bile ducts and colon, should be clarified by a physician at an early stage.
Treatment and therapy
A cure for primary sclerosing cholangitis is not yet possible. Thus, therapy serves to improve the course of the disease and enhance the patient’s quality of life. It is also important to counteract complications. In order to suppress the harmful bile acid, the patient receives ursodeoxycholic acid (UDCA), which is administered on a long-term basis. This is a bile acid that is marginally produced by humans. However, the benefit of ursodeoxycholic acid could not be precisely demonstrated because the mechanism of action could not be established.Bile duct endoscopy offers the possibility of rectifying narrowing of the bile ducts, which improves the drainage of bile. In some cases, liver transplantation may also be necessary, for example if cirrhosis of the liver is already present. In this procedure, the diseased liver is removed and replaced with a healthy donor liver.
Prevention
Preventive measures against primary sclerosing cholangitis are not known. To counteract sequelae such as an intestinal or biliary tumor, regular specialist examinations should be performed.
Follow-up
Patients suffering from primary sclerosing cholangitis should also closely monitor symptoms during follow-up. For a successful healing process, it is important that they keep their doctor’s appointments and have regular checkups. The examinations during follow-up care also serve to check the medication regimen. In any case, the drugs must be taken exactly as prescribed to relieve pain and discomfort in the upper abdomen. If patients conscientiously follow the doctor’s recommendations, they can live transplant-free for a longer period of time. Good self-control is also very useful in preventing episodes of inflammation. Stress is a dangerous trigger, but can be limited by relaxation techniques. Too much alcohol and other stimulants have a negative effect on the body’s defenses and liver function. Therefore, patients should limit their alcohol consumption. Avoiding drugs that damage the liver, such as paracetamol, also reduces the risk of disease. As a preventive and aftercare measure, patients can also take special vitamin preparations to supply their bodies with sufficient nutrients. Regular blood samples can be taken to check how good the liver and bile levels are. In addition, sufferers can ask for the vitamin level in their blood to be determined during their medical examination.
This is what you can do yourself
Primary sclerosing cholangitis causes irregular symptoms in about half of those affected. Because there is no cure for the autoimmune disease to date, symptoms can only be treated symptomatically. As a rule, medication is administered with ursodeoxycholic acid. This achieves an excretion of the body’s own bile acids and counteracts the inflammation of the liver. Upper abdominal discomfort can be reduced as a result. In addition, regular use prolongs transplant-free survival, so conscientious use of the drug is essential. In everyday life, patients should also take care to avoid inflammatory episodes. For example, severe stress should be avoided. To avoid further stressing the liver, heavy alcohol consumption and drugs that damage the liver, such as paracetamol, should be avoided. In an acute inflammatory episode, severe itching can be treated with an antihistamine. In addition, a vitamin deficiency should be remedied by taking appropriate preparations. In this case, it is helpful to have a blood sample taken regularly after consultation with a doctor in order to determine the liver and bile values themselves, as well as vitamin levels in the blood. In general, patients should also visit their physician regularly during symptom-free intervals to monitor the progress of the disease and, if necessary, have examinations such as an ultrasound of the liver or an ERCP examination performed.
