Primary sclerosing cholangitis (PSC) is an inflammatory disease of the liver (hepatic). It probably has an autoimmune origin, that is, linked to the body's defense system (immune system) which, instead of attacking viruses and bacteria, accidentally attacks parts of one's body.
Sclerosing cholangitis leads to progressive scarring (fibrosis) of the bile ducts. The bile ducts carry bile, produced by the liver, to the small intestine, the so-called “small intestine.” In primary sclerosing cholangitis, scar tissue constricts and clogs the bile ducts, causing gradual and severe liver damage (cirrhosis).
The evolution of the disease is very variable but, in most cases, it progresses slowly towards cirrhosis and liver failure, making liver transplantation necessary over a period of 15-20 years.
Disorders (symptoms) begin gradually and include fatigue, itching and, subsequently, yellowing of the eyes, skin and mucous membranes (jaundice). The referring doctor is thehepatologist.
CSP care focuses on controlling (monitoring) the functioning of the liver, managing disorders and, when possible, performing procedures that temporarily open blocked bile ducts.
Although rare, CSP is a major cause of liver transplantation. Liver transplantation can prolong life but the disease, although rarely, can also occur in the transplanted liver.
There is a higher incidence of CSP in males with a male to female ratio of approximately 2: 1. The median age at the time of disease discovery is 40 years.Symptoms
Primary sclerosing cholangitis (CSP) is often diagnosed (diagnosed) before symptoms (symptoms) appear, for example during follow-up blood tests or after an X-ray taken for other reasons showing abnormal liver images .
Early ailments often include:
The progression of the disease is individual and it cannot be predicted whether it will be slow or whether and how fast it will be.
Signs and disorders that may appear later include:
- pain in the upper right side of the abdomen
- night sweats
- enlarged liver
- enlarged spleen
- weight loss
- yellow eyes and skin (jaundice)
As the disease progresses, cirrhosis disorders will develop:
- high blood pressure in the portal vein, the vein that carries blood from the intestine to the liver (portal hypertension)
- accumulation of fluidin the abdomen (ascites)
- severe hepatic insufficiency
In case of severe generalized, lasting and unexplained itching, as well as in case of extreme tiredness for no apparent reason, it is advisable to consult your doctor.
In the event that the person is suffering from ulcerative colitis or Crohn's disease, both inflammatory bowel diseases, it is particularly important to bring to the doctor's attention disorders such as fatigue and unexplained itching since, often, people with primary sclerosing cholangitis have simultaneously one of these diseases.Causes
It is unclear what causes primary sclerosing cholangitis (CSP). It is probably an autoimmune disease, ie linked to the body's defense system (immune system) which when activated by an infection or a toxin, instead of attacking viruses or bacteria, attacks its own tissues or organs by mistake.
Factors that can increase the risk of primary sclerosing cholangitis include:
- age, CSP can develop at any age but is often found between the ages of 30 and 50
- sex, CSP affects men more often
- inflammatory bowel disease, a large percentage of people with CSP also suffer from inflammatory bowel disease
- geographic location, the population of northern Europe has a higher genetic predisposition and a higher risk of developing primary sclerosing cholangitis
A large percentage of people with primary sclerosing cholangitis also suffer from inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn's disease. However, primary sclerosing cholangitis and inflammatory bowel disease do not always appear at the same time. Primary sclerosing cholangitis can occur years before inflammatory bowel disease.
In the case of a diagnosis of primary sclerosing cholangitis, it is important to check whether an inflammatory bowel disease is also present because in this case it can increase the risk of colon cancer. Rarely, people with primary sclerosing cholangitis develop inflammatory bowel disease after liver transplantation.Diagnosis
Tests and procedures used to diagnose primary sclerosing cholangitis (CSP) include:
- blood tests to evaluate liver function, including liver enzyme levels, can provide the physician with clues to ascertain CSP
- serum search for antibodies directed against the perinucleolar region of neutrophils (pANCA), present in a variable percentage from 26-85%, can be strongly indicative of the presence of the disease
- magnetic resonance cholangiopancreatography (MRCP), a test that uses magnetic resonance imaging (MRI) to obtain images of the bile ducts and pancreatic duct. It is the most reliable examination (elective examination) to ascertain primary sclerosing cholangitis and to distinguish it from other causes of obstruction of the bile ducts
- endoscopic retrograde cholangiopancreatography (ERCP), is an x-ray of the bile ducts taken with contrast medium injected directly into the ducts with an endoscope. This test is rarely used for primary sclerosing cholangitis due to the risk of complications from the contrast agent.
- liver biopsy, the doctor takes a small sample of liver tissue with the help of a needle. Liver biopsy helps determine the extent of liver damage and is performed only when the diagnosis of CSP is still uncertain after the less invasive tests have been performed
Primary sclerosing cholangitis (CSP) treatment (therapy) focuses on managing complications and managing liver damage over time. Many drugs have been studied to treat CSP but, to date, none have been found to slow or reverse the progressive liver damage associated with the disease.
Therapies are aimed at relieving ailments (symptoms):
- ursodeoxycholic acid (UDCA), also known as ursodiol, is a naturally occurring bile acid that reduces itching by helping bile to be absorbed
- sequestrants of bile acids, are drugs that bind to bile acids in the intestine avoiding their return to the liver. They are the first choice treatment for itching in primary sclerosing cholangitis
- antihistamines, drugs that help reduce the itching caused by primary sclerosing cholangitis but can worsen some conditions such as dry eyes and mouth
- antibiotics, the obstruction of the bile ducts causes frequent bacterial infections (bacterial cholangitis). To prevent and treat them, people with primary sclerosing cholangitis can be treated with repeated courses of antibiotics.
- nutritional support, primary sclerosing cholangitis makes it difficult to absorb some vitamins and it is therefore necessary to supplement the diet with fat-soluble vitamins (vitamins A, D, E and K)
Treatment for obstruction of the bile ducts
The obstruction of the bile ducts may be due to the progression of the disease but may also indicate a tumor of the ducts. There endoscopic retrograde cholangiopancreatography (ERCP) can help determine the cause of the obstruction which can be treated with:
- dilation of the duct with stent, a procedure that allows the bile ducts to be dilated to allow the passage of bile.Biliary stenting involves the insertion of a small plastic tubular prosthesis which, by supporting the wall of the bile ducts at the level of a narrowing, allows it to be eliminated and prevented from re-forming. Its positioning takes place using an endoscope and the instruments connected to it. In some cases the affected bile duct is dilated by inflating a balloon inside it before inserting the stent
- surgery, used to clear the occluded parts of the duct and reconnect the healthy portions
Liver transplantation, with a healthy liver from a donor, is the only cure for primary sclerosing cholangitis and is reserved for people with liver failure or other serious complications. Although not common, it is possible that primary sclerosing cholangitis also occurs in the transplanted liver.Complications
Complications of primary sclerosing cholangitis can include:
- liver disease and failure, chronic inflammation of the bile ducts in the liver can lead to scarring of tissues (fibrosis, cirrhosis), death of liver cells and, ultimately, loss of liver function
- repeated infectionsFrequent infections in the bile ducts can occur if the scarring of the bile ducts slows or stops the flow of bile. The risk of infection is particularly high after undergoing a surgical procedure to expand a severely occluded bile duct
- portal hypertension, the portal vein it is the main route of blood flow from the digestive system to the liver. The increase in pressure of the portal vein can cause fluid to leak from the liver into the abdominal cavity (ascites) or can divert blood from the portal vein to other veins, causing them to swell (varices) and bleed
- osteoporosisprimary sclerosing cholangitis can cause bone density to decrease (osteoporosis). To prevent osteoporosis, the doctor may prescribe calcium and vitamin D supplements
- bile duct tumorprimary sclerosing cholangitis increases the risk of developing cancer of the bile ducts or gallbladder
- colon cancer, primary sclerosing cholangitis associated with inflammatory bowel disease increases the risk of colon cancer
After the assessment (diagnosis) of primary sclerosing cholangitis, you need to take care of your liver. It is suggested to:
- do not drink alcohol
- get vaccinated against hepatitis A and B
- beware of chemicals in the home and at work
- maintain an optimal weight
- check directions before taking medications, even those over the counter. Make sure your pharmacist and doctor know about liver disease
- do not take supplements that have not been prescribed by your doctor
Mayo Clinic. Primary sclerosing cholangitis (English)
Humanitas Research Hospital. Primary sclerosing cholangitisIn-depth link
Humanitas Research Hospital. Biliary stenting