Primary sclerosing cholangitis is a condition in which the bile ducts inside and outside the liver are inflamed. In primary sclerosing cholangitis, several bile ducts are inflamed, both the large external bile ducts and the small bile ducts inside the liver. Primary means that the cause of the condition is unknown. Sclerosing means narrowing of the bile ducts. These constrictions cause inflammation of the bile ducts, which sometimes leads to constipation. When bile ducts become completely blocked, bile stasis (cholestasis) occurs in the liver. The toxins contained in the bile fluid accumulate in the liver, causing liver damage. In the end, liver cirrhosis may occur in some patients.

Symptoms and causes

Cause

There is a suspicion that PSC is an autoimmune disease. An autoimmune disease is a disease of one's own immune system (defence system). The immune system produces antibodies against the body's own cells, in this case against the tissue of the bile ducts. PSC is more common in people with chronic intestinal inflammation (colitis ulcerosa) and/or autoimmune hepatitis.

Symptoms

In the beginning, this disorder causes little or no symptoms. When inflammations occur in the bile ducts, the flow of bile fluid can be hindered. Cholestasis and/or inflammation of the bile ducts can cause the following symptoms: fatigue, itching, jaundice, fever attacks, pain in the upper right abdomen and fatty diarrhoea

Diagnosis and treatment

Diagnosis

In the first instance, a blood test of the liver values is usually carried out. Liver tests have increased, mainly those pointing in the direction of reduced bile fluid drainage. Subsequently, abnormalities of the bile ducts can be visible on an ultrasound or an MRI scan of the bile ducts (MRCP). So called ‘autoantibodies’ in the blood can also be determined, such as p-ANCA. Due to the lack of bile in the intestine, the patient can have fatty diarrhoea, which means that vitamins, especially vitamin D, are not properly absorbed. This can be found out with a blood test. A liver biopsy is often not necessary to diagnose PSC.

Fortunately, there are also a number of new medications under development for PSC, e.g. 'A randomised, double-blind, placebo-controlled Phase 3 study assessing the safety, tolerability and efficacy of GS-9674 in non-cirrotic subjects with primary sclerosing cholangitis (GS-US-428-4194 Gilead, also known as Cilofexor). Cilofexor aims to reduce scarring and inflammation in the liver (PRIMIS study)'.

Treatment

There is no good treatment for PSC. The drug ursodeoxycholic acid appears to have a beneficial effect on the course of the disease, but the results are not unequivocally positive. Ursodeoxycholic acid is a gallic acid that occurs naturally in bile fluids and does not cause liver damage. The medicine may slow down the disease process and partially improve liver function. Treatment can also consist of combating symptoms. There are medicines to relieve itching and food supplements to prevent or treat vitamin deficiencies. When the bile fluid cannot flow through properly, the physicians may sometimes peform a ERCP . During an ERCP, a narrowing of the bile ducts can be widened (dilatation) or a tube (stent) can be placed in the bile ducts to improve the flow of bile fluid. In case of very serious symptoms and/or liver cirrhosis, it is sometimes necessary to consider a liver transplant.

Source: https://www.mlds.nl/.

Treatment centres and specialisations

Digestive Centre

Latest publication date: 21/01/2021
Supervising author: Dr Vanderstraeten Erik