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This disease, which is characterized by progressive destruction and narrowing of the bile ducts, often coexists with an inflammatory bowel disease (See Inflammatory bowel diseases, ulcerative colitis and Crohn’s disease). Cirrhosis and biliary tract cancer (cholangiocarcinoma) may develop during the natural course of the disease.
PSK although it is thought to be an immune system disease, the etiology and pathogenesis of the disease are not fully known. Toxins, infections, ischemia (decrease in liver blood supply), genetic predisposition and autoimmune factors are among the reasons accused.
A concomitant inflammatory bowel disease (especially ulcerative colitis) is present in 70% of patients with PSC. This relationship between the two diseases suggested that chronic cholangitis may develop as a result of bacterial products that can easily pass through the inflamed colon mucosa or toxic bile acids reaching the liver and biliary tract.[/caption]
There is a familial predisposition in PSK. PSC is more common in families with other autoimmune diseases than in those without. HLA relationship has been shown with HLA B8, DR3 and DR4. The frequency of presence of different autoantibodies in the blood of patients with PSK has increased.
The most common autoantibodies are antinuclear cytoplasmic antibody (ANCA) (65-85%), anticardiolipin antibody (ACA) (65%), and antinuclear antibody (ANA) (53%). Serum complement levels are high in at least 80% of the cases. Traumas in the biliary arterial system and vascular damage that may occur after liver transplantation may cause a picture similar to PSC.
There is no characteristic autoantibody test that can be used in PSK. Blood tests show significant increases in serum alkaline phosphatase (AF) and gamma glutamyl trasferase (GGT) levels, while slight increases in aminotransferase levels (ALT and AST). In cases with jaundice, serum bilirubin levels are found to be high (See Liver function tests). Hypergammaglobulinemia (especially IgM) is seen in 1/3 of the cases. Antinuclear cytoplasmic antibody (ANCA) is positive in blood in 65-85% of cases.
The diagnosis is made by observing typical findings in a cholangiography to be performed in the presence of clinical and biochemical findings and by distinguishing other diseases that may produce similar findings. Cholangiography (radiological imaging of the biliary tract) is performed with the ERCP procedure and is the most valuable diagnostic method used in the diagnosis of PSC (See ERCP- Endoscopic Retrograde Cholangio Pancreatography). In cases where ERCP cannot be performed or is undesirable, MRCP is helpful in the diagnosis (MRCP- Magnetic Resonance Cholangio Pancreatography).
Since technological advances in recent years have enabled better quality images to be obtained with MRCP, MRCP has become the first choice method for diagnosis in many patients. ERCP and MRCP provide information about the extent of the disease and the location and degree of narrowing of the biliary tract. In PSC, both intrahepatic (intrahepatic) and extrahepatic (extrahepatic) bile ducts are commonly involved. In ERCP and MRCP, narrowing of the bile ducts, enlargement in the form of beads, and the appearance of dried-pruned trees are characteristic findings for PSK.
A liver biopsy (taking a tissue sample from the liver with a fine needle) is rarely needed for diagnosis. Biopsy is helpful in staging the disease and determining the prognosis. The most common characteristic finding in liver biopsy is fibrosis (connective tissue development) that resembles an ‘onion skin’ appearance around the bile ducts.
In up to 10% of patients without an abnormality in cholangiography, the appearance of onion skin can be detected in the small bile ducts in the liver biopsy. This condition is called small duct Primary sclerosing cholangitis (small-duct PSC). This type of the disease has a better prognosis than the classical type.
Primary biliary cirrhosis, secondary sclerosing cholangitis, diffuse liver metastasis, cholangiocarcinoma, extensive liver abscesses, amyloidosis, congenital biliary tract diseases, graft versus disease (a picture that occurs as a result of the recipient rejecting the donated marrow cells after organ transplantation), after liver transplantation and autoimmune pancreatitis Findings similar to primary sclerosing cholangitis may occur in the liver. Differential diagnosis is made by using appropriate biochemical, immunological and radiological methods.
The disease is most common between the ages of 25-45. 70% of cases are associated with IBD. Most of the patients do not have any symptoms of the disease (Asymptomatic patient).
The diagnosis is usually made by the doctor’s suspicion during the investigation of abnormalities in liver function tests requested at the time of admission to the outpatient clinic or during routine examinations of a patient with inflammatory bowel disease.
The most common findings are abdominal pain in the right upper quadrant, fatigue, itching and weight loss. Some cases may remain asymptomatic for many years. In some asymptomatic cases, the disease can be seen histologically and radiologically. When symptoms appear, the disease is mostly advanced.
The most common findings on physical examination are jaundice and liver enlargement (hepatomegaly).Hastaların 1/3 inde dalakta da büyüme saptanır (splenomegali).
As a result of insufficient bile flow to the intestine (chronic cholestasis), fat digestion is disrupted and the absorption of fat-soluble vitamins (vitamins A, D, E and K) from the intestines decreases. Fatty stools and vitamin A, D, E and K deficiency occur in patients.
Deficiencies of these vitamins cause visual disturbances, osteoporosis and bleeding. A serious complication of PSK is the development of cancer in the biliary tract (cholangiocarcinoma).
Cholangiocarcinoma should be considered in significant narrowing of the large bile ducts in a patient with PSC and necessary investigations should be performed for diagnosis. Autopsy studies have shown that up to 1/3 of patients with PSC may develop asymptomatic cholangiocarcinoma in the bile ducts in the liver.
There is no effective medical treatment for PSK. UDCA (Ursodeoxycholic acid), a highly effective drug in the treatment of primary biliary cirrhosis, is generally ineffective in the treatment of PSC.
Although some improvement in biochemical values can be achieved with UDCA treatment, it does not have a positive effect on disease progression rate, survival time and delaying liver transplantation.
The recommended dose of UDCA for treatment is 10-15/mg/kg/day. The most common side effect of the drug is diarrhea. When the deficiency of the above-mentioned vitamins is detected, these vitamins are given externally and an attempt is made to close the deficit.[/caption]
Stenosis in the bile ducts can be expanded with a balloon by performing ERCP and a stent can be placed in the narrow area. Liver transplantation is the only effective treatment for patients with advanced PSC and cholangiocarcinoma of the biliary tract. Three-year survival after transplantation in PSC is between 85-90%. The development of stenosis in the biliary tract after transplantation is a common problem. PSC develops again after transplantation in 10-20% of cases.
