Primary sclerosing cholangitis (PSC) is characterized by patchy inflammation, fibrosis and strictures of bile ducts, the cause is not known. 80% of patients have an inflammatory bowel disease, ulcerative colitis most often simultaneously. Other associated physical states are connective tissue diseases, autoimmune disorders and immune deficiencies that are sometimes complicated by opportunistic infections. Fatigue and itching develop insidiously and are progressive. Diagnosis is made by cholangiography (magnetic resonance cholangiopancreatography [MRCP] or ERCP). In advanced cases, liver transplantation is indicated.

(See also Overview biliary function.)

Primary sclerosing cholangitis (PSC) is characterized by patchy inflammation, fibrosis and strictures of bile ducts, the cause is not known. 80% of patients have an inflammatory bowel disease, ulcerative colitis most often simultaneously. Other associated physical states are connective tissue diseases, autoimmune disorders and immune deficiencies that are sometimes complicated by opportunistic infections. Fatigue and itching develop insidiously and are progressive. Diagnosis is made by cholangiography (magnetic resonance cholangiopancreatography [MRCP] or ERCP). In advanced cases, liver transplantation is indicated. (See also Overview biliary function.) PSC is the most common form of sclerosing cholangitis. Most (70%) of patients with PSC are men. The mean age at diagnosis is 40 years. Etiology The cause of the disease is unknown, but it is an association of PSC with inflammatory bowel diseases with chrnische. Such is also present in 80% of PSC patients. Conversely develop about 5% of patients with ulcerative colitis, and about 1% of those with Crohn’s disease, a PSC. This association and various autoantibodies (z. B. smooth muscle antibodies and perinuclear antineutrophil antibodies [pANCA]) suggest immune-mediated mechanisms. T cells appear to be involved in the process of destruction of the bile ducts, so that a disruption of cellular immunity appears to be. In addition, there may be a genetic predisposition, as the fault occurs family and with higher incidence in carriers of HLA-B8 and HLA-DR3, both of which are associated with autoimmune diseases. Possibly a previously unknown trigger causes (eg., Bacterial infection, Gallengangsischämie) in genetically predisposed individuals the PSC. Symptoms and signs The onset is usually insidious with increasing fatigue and later also itching. Jaundice occurs late. About 10-15% of patients have repeated episodes of pain in the right upper abdomen and fever – possibly due to ascending bacterial cholangitis. Steatorrhea and a lack of fat-soluble vitamins can develop. A persistent jaundice indicates advanced disease. In about 75% of patients with symptomatic gallstones and choledocholithiasis develop. Some patients remain asymptomatic until late stages and shows in the diagnosis already hepatosplenomegaly or cirrhosis. PSC is progressing slowly but surely, as a rule. In the final stage of the disease is decompensated cirrhosis, portal hypertension, ascites and liver failure. The period between the initial diagnosis and the liver failure is approximately 12 years. Although there is a strong association between the PSC and inflammatory bowel disease, the two diseases are different courses. An ulcerative colitis may occur years before the PSC and seems to take a less severe course if it is associated with a PSC. Similarly, the colectomy does not change the course of the PSC. The simultaneous presence of PSC and inflammatory bowel disease increases the risk of developing colorectal cancer, regardless of whether a liver transplant was performed or not. A cholangiocarcinoma develops in 10-15% of patients. Diagnostic magnetic resonance cholangiopancreatography (MRCP) The suspected PSC arises in patients with unexplained abnormal liver function, especially in patients who suffer from a chronic inflammatory bowel disease. Typical is a cholestatisches pattern with increased alkaline phosphatase and gamma glutamyl transferase (GGT) and less highly elevated transaminases. Gamma globulin and IgM are often increased. Smooth muscle antibodies and p-ANCA are sometimes positive. In contrast, anti-mitochondrial antibody (AMA), which appear typical for primary biliary cirrhosis, characteristically negative. First, an imaging method for representing the hepatobiliary system to exclude extrahepatic closure, the ultrasound is used. Although ultrasound or computed tomography can show bile duct extensions, the diagnosis requires cholangiography so multiple stenoses and extensions in the intra- and extrahepatic bile ducts are confirmed. Cholangiography should begin with the magnetic resonance cholangiopancreatography (MRCP). ERCP is the second choice in general because it is invasive. A liver biopsy is usually not necessary for the Diagsosestellung. If you performed, it shows proliferation of small bile ducts, periductal fibrosis, inflammation and loss of bile ducts. With progressive disease, the periductal fibrosis spreads from the portal regions forth and eventually leads to biliary cirrhosis. Measurement of serum tumor markers and ERCP monitoring with brush cytology should be performed regularly in order to eliminate a biliary tract carcinoma. Therapy Supportive treatment ERCP dilation in dominant stenoses transplantation for recurrent bacterial cholangitis, cachexia or decompensated cirrhosis. Asymptomatic patients have to merely monitor (physical examination, liver function tests twice a year and imaging once a year). Ursodeoxycholic acid (eg., 5 mg / kg p.o. 3 times a day up to 15 mg / kg / day) reduced the itching and improved the biochemical markers, but not survival. Chronic cholestasis and cirrhosis require supportive treatment. Episodes of bacterial cholangitis justify antibiotics and therapeutic ERCP as needed. If a single stenosis as the main cause for a closure comes into consideration (a predominant constriction is found in about 20% of patients) can ERCP dilation better (with brush cytology or biopsy to exclude malignant process) and optionally short-term stenting symptoms , Liver transplantation is the only treatment that improves the life expectancy in patients with PSC and may be curative. Indications for transplantation are recurrent bacterial cholangitis, cachexia or complication in end-stage liver disease (eg. B. untreatable ascites, portosystemic encephalopathy, bleeding esophageal varices). Important Points Most patients (80%) with PSC have an inflammatory bowel disease, usually an ulcerative colitis, and many have autoantibodies. PSC should be adopted especially in patients with inflammatory bowel disease that have an inexplicable pathological patterns in the liver values. Extrahepatic biliary obstruction should be excluded by ultrasound, then it should be carried out MRCP (or, as a second choice, ERCP). Patients will be monitored through regular monitoring of liver enzymes and treating symptoms and complications (eg. As ERCP for widening dominant strictures). Liver transplantation should be considered in recurrent cholangitis or complications of liver failure into consideration.

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