Primary sclerosing cholangitis abbreviated to PSC , and primary cholangitis ,sclerosing formerly known as first biliary cirrhosis , are two forms of biliary liver diseases that lead to cirrhosis and liver failure .
- But they are two separate diseases called cholestasis ; due to poor flow of bile through the liver and bile ducts .
- You can conclude from this term cholangitis ; It is inflammation of the bile ducts , and these bile ducts can be inside or outside the liver .
- Bile is normally produced by hepatocytes in the liver, and travels through the intrahepatic bile ducts , and additional hepatic ducts into the intestine to aid in the digestion , and absorption of fats.
- In both primary sclerosing cholangitis and primary cholangitis , increased resistance to bile flow occurs , causing bile to accumulate in the liver resulting in chronic inflammation and eventually cirrhosis .
- There are many bile ducts in the biliary tree , and what happens in the PSC is essentially a system of inflammation , fibrosis , scarring , and strictures . Then there are dilatations , and then again narrowings like this and so on , and this appearance is known as beads .
- It is often referred to by this name , because it comes from a restriction which means narrowing , and thus an obstruction to the flow of bile . It is more common in males than in females , between the ages of 30 and 40 .
- Primary cholangitis, is also a chronic condition , and progressive in this case ; It is a true autoimmune condition that leads to the destruction of the small bile ducts , within the liver resulting in primary cholestasis which is not uncommon , such as primary cholangitis .
- primary cholangitis has a prevalence of 35 per 100,000. The female to male ratio is 9 to 1 and this is often seen in middle age to highlight this, and it is believed that about 1 in 1,000 women over the age of 45 has the condition.
- As we said that primary cholangitis, considered an autoimmune condition, is more common in females and also has associations with other autoimmune diseases such as celiac disease, and hypothyroidism.
- It is believed that lower immune tolerance in middle age leads to targeting of youngsters . Interlobular bile ducts are thought to be destroyed by the immune system, progressively deteriorating bile flow resulting in chronic inflammation in cholestasis , and then specifically cirrhosis .
- Mitochondrial antibodies are thought to target lipoic acid , particularly pyruvate dehydrogenase E2 on epithelial cholangiocytes .
- Although primary cholangitis is a progressive disease , there are some cases in which progress is minimal , in some cases the opposite , but on average the final stages reach 15 to 20 years for diagnosis .
primary sclerosing cholangitis causes
- Now why does this happen what is the reason for it? There is a strong link between primary sclerosing cholangitis , and inflammatory bowel disease in particular ulcerative colitis .
- Approximately eighty percent of IBD sufferers from PSC , although the exact pathophysiology is not currently known , but it is an immune condition , not a true autoimmune condition .
- Because in IBD there are autoantibodies , and these autoantibodies are responsible for the immune destruction of the bile ducts , that’s the really big link between IBD and PSC .
- There is a genetic predisposition in particular , which is more prevalent in patients with HLA B2 and Dr3 subtypes , and patients are also thought to have a disorder of the bile duct epithelium .
- Due to the close relationship with IBD, it has been hypothesized that there is movement of bacteria from the gut , into the portal system resulting in a systemic inflammatory response .
- They then lead to disruption of the biliary epithelium causing the molecules to be exposed to bile acids , and thus the outflow of bile from the liver is decreased .
- Which means more bile remains in the liver which leads to more chronic inflammation , and scarring which gives a vicious cycle with the flow of bile constantly being blocked which eventually leads , on average , to cirrhosis of the liver .
- The survival rate is about 15 years from diagnosis , which usually reaches biliary cirrhosis by about that time .
- A possible complication of primary sclerosing cholangitis is cholangiocarcinoma , a primary malignancy of the bile ducts . It occurs in about 1 in 5 patients and is characterized by a poor prognosis .
- There is also an increased risk of other hepatocellular carcinomas including hepatocellular carcinoma , malignant tumors of the gallbladder as well as colorectal , pancreatic and rectal cancers .
primary sclerosing cholangitis symptoms
- This is asymptomatic, and the condition is discovered incidentally .
- but the most common symptom is fatigue , and other symptoms such as the classic triad of “fatigue, itching, fever or pruritus, jaundice”, and nonspecific upper abdominal pain.
- Jaundice, or yellow discoloration of the skin or mucous membranes, is most commonly caused by a buildup of bilirubin in the body.
- Another possible characteristic that patients may also have a deficiency in fat-soluble vitamins , can present directly with signs of cirrhosis in primary sclerosing cholangitis .
sclerosing cholangitis and primary cholangitis diagnosis
- (1) – Simple blood test “alkaline phosphatase level” alq foss i
- (2) – autoantibodies
- (3) – Mitochondrial antibodies
- (4) – Magnetic resonance imaging of the pancreas and cholangiocarcinoma “MRCP”
- (5) – Endoscopic retrograde cholangiopancreatography (ERCP)
- (1) – A simple blood test is the alkaline phosphatase level “ALQ Foss I”, which is a very important laboratory test .
Where alkaline phosphatase is elevated , liver function tests can also be elevated, but they can also be interestingly normal, but ALQ FOSs I, is almost always elevated .
The diagnostic criteria for primary biliary cholangitis require , an elevation of alkaline phosphatase 1.5 times the upper limit of normal histological evidence .
- (2) – autoantibodies :
Autoantibodies can be present in 97 percent of cases , particularly anti-smooth muscle , or antinuclear antibodies in 75 percent of cases , and anti-neutrophil cytoplasmic or p-anchor antibodies .
- (3)- Mitochondrial antibodies :
Mitochondrial antibodies are positive in 90 percent of PCH cases, and this is a high-specificity marker of antinuclear antibodies present in about 50 percent of cases.
There are two other very expensive tests with big long names, and this is recommended for differentiating between primary sclerosing cholangitis and primary cholangitis.
- (1) – First, “Magnetic resonance cholangiopancreatography” (MRCP) :
which is magnetic resonance imaging of the bile ducts and pancreas . It is an imaging test that allows to see the hepatobiliary system , and it is considered the best test for diagnosis .
The distinctive result is strictures separated by normal parts of the bile ducts , ie the appearance of beads as mentioned above , in the case of primary sclerosing cholangitis .
- (2) – The second option is ‘ERCP’ which is endoscopic hereditary cholangiography :
which is also a very important but slightly more invasive test.
primary sclerosing cholangitis treatment
- (1) – Effective treatment of primary sclerosing bile duct transplants is performed in the liver , because it improves mortality.
The recurrence of primary sclerosing cholangitis , however, is still a risk after liver transplantation .
Liver transplantation is the only treatment that improves life expectancy.
- (2) Use of cholestyramine :
Cholestyramine is a form of bile acid , it binds to the bile fluid in the digestive tract to prevent its re-absorption .
Where an insoluble compound is formed in the intestine , it is eliminated in the feces . Cholestyramine is used to relieve symptoms such as itching , and as immunosuppressants .
- (3) – Ursodeoxycholic acid :
Ursodeoxycholic acid is sometimes prescribed , which improves liver function tests . However , studies have shown that it has little or limited benefit from the use of the drug , in terms of survival or symptoms .
Primary cholangitis often has a good response to ursodeoxycholic acid , and it is believed that when it is incorporated into the bile it reduces toxicity to the biliary epithelium, thus reducing inflammation.
- (4) – Opticolic acid :
About 40 percent of cases are resistant to pseudoxacolic acid, and therefore it is replaced by obeticholic acid . It is a relatively new synthetic bile acid , to be used if ursodeoxycholic acid has failed or is not tolerated .
It is a vanadoid X receptor agonist which when activated reduces bile acid synthesis and increases clearance , as well as reducing inflammation symptoms such as inflammation and pruritus .
- (5) – Anti-fibrotic drugs such as cyclosporine, tacrolimus, and methotrexate, did not show consistent clinical improvement in these patients .