Sclerosing cholangitis is a chronic, fibrotizing and constrictive inflammation of the biliary ducts that progresses slowly and leads to cholestatic liver damage, to cirrhosis and in some patients to cholangiocarcinoma
E.g. in the Nordic countries, the prevalence of sclerosing cholangitis is particularly high and it is the most common indication for liver transplantation.
MRI of the liver is ordered at a lower threshold than normal on a patient with IBD Ulcerative ColitisCrohn's Disease who has fluctuation in the liver test results.
Cholangitis with fever is possible. Weight loss and jaundice are symptoms of a late phase.
Endoscopic retrograde cholangiography (ERCP) is the "golden standard": confirmation of the diagnosis, samples from the biliary tract, concurrent dilatation and stenting of the biliary ducts if needed.
Liver biopsy: in case of non-specific histology, in the differential diagnostics of diseases of the small biliary ducts or other chronic hepatitides, gradation of the inflammation and fibrosis
Colonoscopy to detect silent colitis is indicated in all patients.
Autoimmune cholangitis is a rarer diagnostic alternative.
The patient often has other autoimmune diseases; IBD in less than 10%
Diagnosis: MRI; serum IgG4 increased; typical histology in the papilla/bile ducts, IgG4-positive plasma cells
Good response to glucocorticoid therapy
Complications
Strictures of the biliary tract
Increased risk for gallstones and cancer of the gallbladder.
Cholangiocarcinoma develops in 10-20% of the patients.
Cirrhosis develops at the end stage of the disease.
Ursodeoxycholic acid is commonly used, 15-20 mg/kg/day divided into 2 doses. When started in time, ursodeoxycholic acid slows the progress of inflammation and corrects liver biochemistry to a certain extent but the evidence on clinical benefit is scarce.
Metronidazole (600-800 mg/day) may retard the progression of the disease. The drug is used if there is no response to ursodeoxycholic acid.
Biliary strictures can be dilatated and stented endoscopically (ERCP).
Management of complications
Cholangitides are treated with antibiotics (often with fluoroquinolones).