Primary sclerosing cholangitis is a chronic inflammation of the bile ducts. It causes scarring, hardening, which narrows the bile ducts.
What is primary sclerosing cholangitis?
Primary sclerosing cholangitis (PSC) is a special form of cholangitis (inflammation of the bile ducts). It is classified as an autoimmune disease. As part of the disease, those affected suffer from progressive and chronic bile duct inflammation, which occurs both inside and outside the liver.
Primary sclerosing cholangitis is closely associated with other autoimmune diseases such as ulcerative colitis, an inflammatory bowel disease. As the autoimmune disease progresses, it leads to scarring of the bile ducts. This in turn leads to bile stasis. In the worst case, cirrhosis of the liver can develop, for the treatment of which a liver transplant is necessary. See healthknowing for What is the Definition of CAR.
Primary sclerosing cholangitis is relatively rare. It is estimated that 1 to 5 out of 100,000 people suffer from it. It occurs in men two or three times as often as in women. People between the ages of 30 and 50 are particularly affected. Ulcerative colitis is also present in 70 out of 100 patients, and in some cases also Crohn’s disease.
This disease is also a chronic inflammatory bowel disease. In addition, other autoimmune diseases such as Sjögren’s syndrome or autoimmune hepatitis can also present simultaneously with primary sclerosing cholangitis.
Causes
It is still unclear what causes primary sclerosing cholangitis. In some families, it is more common among first-degree members. Therefore, doctors 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. Faulty immune reactions in the bile duct mucosa lead to the development of primary sclerosing cholangitis. However, microorganisms in the digestive region such as bacteria should also play a role.
Symptoms, Ailments & Signs
In the early stages of primary sclerosing cholangitis, there are no symptoms, so the disease usually goes unnoticed for a long time. Complaints only appear when the damaged bile ducts restrict the functions of the liver. In this case, the affected person suffers from agonizing itching, weight loss, exhaustion, tiredness and a yellowish discoloration of the skin and mucous membranes, which doctors also call icterus (jaundice).
In addition, the patient is sensitive to pressure and suffers from pain in the upper right abdomen. Most patients also have ulcerative colitis, a chronic inflammatory bowel disease. Furthermore, the risk of bacterial inflammation occurring in the bile ducts increases. Such bacterial cholangitis manifests itself in flares and causes abdominal pain on the right side of the abdomen, weakness and fever.
Diagnosis & course of disease
If primary sclerosing cholangitis is suspected, the patient should consult a gastroenterologist or an internist who specializes in treating such conditions. The first step of the examination is the recording of the medical history (anamnesis).
The doctor asks the patient if they have cramping or constant abdominal pain, have ulcerative colitis, have a history of gallstones, or have a fever. The medical history is followed by the physical examination, during which the doctor examines the patient’s skin. The yellowish discoloration of the skin is an indication of liver damage.
The doctor listens to the bowel sounds with a stethoscope. In this way he controls the stool and air content of the intestine. However, primary sclerosing cholangitis cannot always be detected by physical examination. For this reason, further investigations are taking place. This includes 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 used to monitor the bile ducts and liver, with the help of which primary sclerosing cholangitis can be differentiated from other diseases. If the suspicion of PSC is confirmed, a more detailed examination of the bile ducts must be carried out, which is done with magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP).
The ERCP can also be used therapeutically. The course of primary sclerosing cholangitis varies from person to person. If the chronic inflammation progresses, there is a risk of consequential damage such as fibrosis, in which the connective tissue proliferates abnormally, 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 take an individual and extremely different course. Therefore, in some cases, there is a risk of serious complications. However, these do not affect every individual patient. A common consequence of primary sclerosing cholangitis is bile duct obstruction.
Because of the narrowing, there is an increased risk of gallstones forming over the narrowing. Bacterial bile duct infection (acute cholangitis) can also occur, which is manifested by symptoms such as colicky pain, fever and chills.
One of the serious complications of PSC is liver cirrhosis. It is caused by chronic accumulation of bile in the liver. Doctors also speak of secondary biliary cirrhosis. A knotty remodeling of the liver takes place, which shrinks and increasingly loses its functions. In such cases, a liver transplant is often necessary.
The primary sclerosing cholangitis also increases the risk of developing cancer. For example, 13 to 14 percent of all patients are at risk of developing hepatobiliary carcinoma (cholangiocarcinoma). At the same time, the risk of colon cancer, liver cell cancer, pancreatic cancer and gallbladder cancer increases. For this reason, patients must have regular cancer screening tests.
Other possible consequences of primary sclerosing cholangitis are rheumatic complaints, osteoporosis (bone loss) and a lack of vitamin A, vitamin D, vitamin E and vitamin K. These vitamins have in common that they have fat-soluble properties.
When should you go to the doctor?
Symptoms such as jaundice, fatigue, and weight loss suggest primary sclerosing cholangitis. A doctor’s visit is indicated if these and other symptoms typical of the disease appear. If there is abdominal pain or the itching that often occurs in the arms and legs, 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 symptoms described appear. People with chronic gastrointestinal complaints are also among the risk groups.
Anyone with a family history of primary sclerosing cholangitis should see a doctor. In addition to the family doctor, the chronic condition can be referred to an internist or a specialist in diseases of the gallbladder. The condition is usually treated as an inpatient in a specialist clinic. In the case of chronic diseases, the patient must consult the doctor closely. The medical professional should be informed of any unusual symptoms so that adjustments in treatment can be made. If cirrhosis of the liver occurs, you must call an ambulance and, if necessary, provide first aid. Any subsequent symptoms such as bone loss or tumors of the bile ducts and the large intestine must be clarified by a doctor at an early stage.
Treatment & Therapy
A cure for primary sclerosing cholangitis is not yet possible. The therapy serves to improve the course of the disease and improve the quality of life of the patient. It is also important to counteract complications. In order to suppress the harmful bile acid, the patient receives ursodeoxycholic acid (UDCA), which is administered over the long term.
This is a bile acid that is produced to a small extent by humans themselves. However, the benefits of ursodeoxycholic acid could not be precisely proven because the mechanism of action could not be fathomed. A bile duct endoscopy makes it possible to correct constrictions in the bile ducts, which improves the flow of bile.
In some cases, a liver transplant 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. In order to counteract secondary diseases such as an intestinal or bile duct tumor, specialist medical examinations should be carried out regularly.
Aftercare
Patients suffering from primary sclerosing cholangitis should also closely monitor the symptoms during follow-up. For a successful healing process, it is important that you keep the doctor’s appointments and have regular check-ups. The examinations as part of the aftercare also serve to check the drug setting.
In any case, the remedies should be taken exactly as directed to relieve pain and discomfort in the upper abdomen. If those affected conscientiously follow the doctor’s recommendations, they can live without a transplant for a long time. Good self-control is also very useful in preventing flare-ups. Stress is a dangerous trigger, but it can be curbed with 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 liver-damaging agents, such as paracetamol, also reduces the risk of disease. As a precaution and aftercare, those affected can also take special vitamin preparations in order to supply their body with sufficient nutrients. By taking blood regularly, you can check how good the values of the liver and bile are. In addition, those affected can ask for the determination of the vitamin level in their blood during the medical examination.
You can do that yourself
Primary sclerosing cholangitis causes irregular symptoms in about half of those affected. Since the autoimmune disease cannot yet be cured, complaints can only be treated symptomatically.
As a rule, the medication is adjusted with ursodeoxycholic acid. This achieves an excretion of endogenous bile acids and counteracts the inflammation of the liver. This can reduce upper abdominal pain. In addition, regular use extends transplant-free survival, so that the medication should be taken conscientiously.
In everyday life, patients should also take care to avoid flare-ups. For example, severe stress should be avoided. In order not to further burden the liver, heavy alcohol consumption and liver-damaging drugs such as paracetamol should be avoided. In an acute episode of inflammation, severe itching can be treated with an antihistamine. In addition, a vitamin deficiency should be remedied by taking appropriate preparations. Here it is helpful to have a blood sample taken regularly after medical consultation, in order to have the liver and bile values themselves, as well as the vitamin levels in the blood, determined.
In general, patients should also visit their doctor regularly in 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 carried out.
