Posts Tagged ‘Progressive’

What are symptoms of primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts.
- This disease channelizes bile from the liver into the intestines.
- Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine, particularly fat.
- This liquid also helps to get rid of the body of toxins.
- Liver cells secrete the bile they make into small canals within the liver.
- The bile flows through the canals and into larger collecting canals (ducts).
- The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts.
- From the extrahepatic bile ducts, the bile flows into the intestine where the bile mixes with food.

In primary sclerosing cholangitis :
- The intrahepatic and extrahepatic bile ducts become inflamed,
- scarred and thickened (sclerotic),
- narrowed,
- finally obstructed.

Obstruction of the ducts can lead to:
- abdominal pain,
- itching,
- jaundice,
- infection in the bile ducts (cholangitis),
- liver scarring that leads to liver cirrhosis,
- liver failure.

Symptoms of primary sclerosing cholangitis:
- Most patients with early primary sclerosing cholangitis have no symptoms.
- The presence of primary sclerosing cholangitis is recognized only because of abnormally elevated blood levels of liver enzymes (particularly alkaline phosphatase levels).
- This is often recognized along with a routine physical examination.
Early symptoms of primary sclerosing cholangitis include
- Fatigue
- bodily itching (pruritus)

With the progress of the disease:
- Patients may develop jaundice (yellowing of skin and darkening of urine).
- Jaundice is due to the accumulation of bilirubin in the body.
- The bilirubin accumulates because it is not able to be eliminated in the bile.
- This is due to prolonged obstruction of the bile ducts.
- The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow.
- It may be due to accumulation of bile salts in the body.
- It is as a result of obstruction of the bile ducts.

As primary sclerosing cholangitis progresses, patients typically develop:
- Right upper abdominal pain,
- Fever,
- Fatigue,
- Pruritus
- Jaundice
- These patients also are at risk of developing primary sclerosing cholangitis complications.
- The patients with the autoimmune form of primary sclerosing cholangitis have more rapid symptoms of abdominal pain, jaundice and fever.

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How is primary sclerosing cholangitis treated?

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts.
- This disease channelizes bile from the liver into the intestines.
- Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine, particularly fat.
- This liquid also helps to get rid of the body of toxins.
- Liver cells secrete the bile they make into small canals within the liver.
- The bile flows through the canals and into larger collecting canals (ducts).
- The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts.
- From the extrahepatic bile ducts, the bile flows into the intestine where the bile mixes with food.

In primary sclerosing cholangitis :
- The intrahepatic and extrahepatic bile ducts become inflamed,
- scarred and thickened (sclerotic),
- narrowed,
- finally obstructed.

Obstruction of the ducts can lead to:
- abdominal pain,
- itching,
- jaundice,
- infection in the bile ducts (cholangitis),
- liver scarring that leads to liver cirrhosis,
- liver failure.

Diagnosis of primary sclerosing cholangitis includes:
- Blood tests
- Radiologic tests
- Colonoscopy

The treatment of primary sclerosing cholangitis includes:
- Cholestyramine (Questran) or rifampin(Rifadin) to diminish itching.
- Antibiotics for infection, specifically cholangitis.
- Vitamin D and calcium to prevent bone loss (osteoporosis).
- Medications such as ursodiol (UDCA) to retard the progression of primary sclerosing cholangitis.
- ERCP with balloon dilatation.
- Stenting (a procedure in which the bile ducts are stretched open) for primary sclerosing cholangitis patients with dominant extra-hepatic duct strictures.
- Liver transplantation for patients with advanced cirrhosis.

Medications
- Ursodiol (UDCA),
- Prednisone,
- Methotrexate(Rheumatrex, Trexall),
- Colchicine,
- 6-mercaptopurine,
- Tacrolimus (Prograf),
- Cyclosporine (Neoral, Gengraf)

UDCA
- Ursodiol (UDCA) is a bile acid.
- This is given orally and replaces other bile acids in the body.
- UDCA is believed to protect against damaging effects of other bile acids on the liver cells.
- It also can induce formation of antioxidants.
- UDCA is the most extensively studied medication for primary sclerosing cholangitis.
- Its standard doses are15 mg/kg/day.
- It has been shown to improve fatigue, and improve blood levels of liver enzymes in these patients.
- Higher doses (20-30 mg/kg/day) UDCA has been shown to improve blood liver enzymes.
- It also decreases bile duct inflammation, and decrease liver scarring.
- UDCA is safe and well tolerated.
- It may improve bile duct disease and retard the development of liver cirrhosis.
- UDCA may decrease colon cancer risk in patients with both primary sclerosing cholangitis and chronic ulcerative colitis.

Treatment of dominant strictures
- Dominant strictures are major narrowing in the extrahepatic bile ducts.
- Dominant strictures of the extrahepatic bile ducts occur in 7%-20% of primary sclerosing cholangitis patients.
- ERCP and balloon dilatation (stretching) of the stricture can improve symptoms and abnormal blood levels of liver enzymes and bilirubin.
- Successful dilatation of dominant strictures decreases the risk of developing cholangitis.
- ERCP and dilatation of dominant strictures should be done in centers with highly experienced physicians.
- In ERCP, doctors often also perform brush cytology of the dominant strictures to exclude cholangiocarcinoma.
- Surgery is another treatment for dominant extrahepatic strictures.
- Surgical resection of the stricture followed by creation of a choledocho-jejunostomy which is an artificial passage for bile formed by attaching the bile duct from above the stricture directly into the small intestine.
- This can improve symptoms, delay liver transplantation, and lower the risk of cholangiocarcinoma.

Liver transplantation
The reasons for liver transplant in primary sclerosing cholangitis patients are similar to those in other forms of end-stage liver disease. They are:
- Internal bleeding due to rupture of esophageal varices.
- Severe ascites that are refractory to medical treatment.
- Frequent episodes of bacterial cholangitis.
- Hepatic encephalopathy.

How is primary sclerosing cholangitis diagnosed?

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts.
- This disease channelizes bile from the liver into the intestines.
- Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine, particularly fat.
- This liquid also helps to get rid of the body of toxins.
- Liver cells secrete the bile they make into small canals within the liver.
- The bile flows through the canals and into larger collecting canals (ducts).
- The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts.
- From the extrahepatic bile ducts, the bile flows into the intestine where the bile mixes with food.

In primary sclerosing cholangitis :
- The intrahepatic and extrahepatic bile ducts become inflamed,
- scarred and thickened (sclerotic),
- narrowed,
- finally obstructed.

Obstruction of the ducts can lead to:
- abdominal pain,
- itching,
- jaundice,
- infection in the bile ducts (cholangitis),
- liver scarring that leads to liver cirrhosis,
- liver failure.

The diagnosis of primary sclerosing cholangitis inclde:
- It is suspected from the symptoms (such as fatigue, itching, and jaundice).
- Abnormal laboratory tests (such as abnormally elevated blood levels of alkaline phosphatase and other liver enzymes).
- It is confirmed by demonstration of abnormally thickened bile ducts using special radiological tests.

It is also important to exclude diseases that can mimic primary sclerosing cholangitis like:
- primary biliary cirrhosis(PBC)
- gallstones in the bile ducts
- bile duct cancers
- strictures

Blood Tests
- The blood level of alkaline phosphates is usually elevated in primary sclerosing cholangitis.
- The blood levels of other liver enzymes (AST and ALT) may also be mildly elevated.
- Except in those patients with the autoimmune form of primary sclerosing cholangitis, the bilirubin usually is normal.
- It gradually increases as the disease progress.
- Antimitochondrial antibody (AMA), which is elevated in patients with PBC, is usually normal in these patients.

Radiologic Tests
- Endoscopic retrograde cholangio-pancreatography (ERCP).
- Magnetic resonance cholangio-pancreatography (MRCP).
- These are commonly performed to visualize the intrahepatic and extrahepatic ducts.
- These ducts are typically normal in appearance in patients with PBC.
- In primary sclerosing cholangitis patients, these ducts have a beaded appearance (multiple narrowings along the ducts with areas of widening in between).
- MRCP is noninvasive and safe.
- ERCP is more invasive and carries a 5%-6% chance of causing an attack of acute pancreatitis.
- However, ERCP has the advantage of obtaining cell samples (a process called brush cytology) from the bile ducts.
- Brush cytology is not very accurate.
- It can help to diagnose cholangiocarcinoma.
- During ERCP, doctor can also insert balloons and stents across major areas of narrowing (dominant strictures).
- This is done to relieve obstruction and treat infection.

Colonoscopy
- Patients with primary sclerosing cholangitis have a high likelihood of also having ulcerative colitis.
- Patients with primary sclerosing cholangitis and ulcerative colitis have higher risks of developing colon cancer.
- Colonoscopy is important to both diagnose ulcerative colitis and for early detection of cancer.
- It is also used to study the precancerous conditions.