View source for Primary sclerosing cholangitis

'''Primary sclerosing cholangitis''' (PSC) is a disorder characterised by progressive chronic inflammation of the intra- and extrahepatic ducts, eventually leading to obliteration of the ducts. Almost all patients also have [[Inflammatory bowel disease (Crohn disease and ulcerative colitis)|IBD]], especially ulcerative colitis. It can progress to [[cirrhosis]] and there is a high risk of development of [[cholangiocellular carcinoma]]. If there is concomitant ulcerative colitis, the risk for [[colorectal cancer]] is also elevated.

== Clinical features ==
PSC may present asymptomatically (only abnormal laboratory parameters) as it remains asymptomatic for a long time. Then, nonspecific symptoms like fatigue and pruritus appear. Later, symptoms of [[cholestasis]] appear, like [[jaundice]], and itching.

Because most patients with PSC also have concomitant IBD, symptoms of IBD can also be present.

== Diagnosis and evaluation ==
As with any [[cholestasis]], [[ALP]], [[GGT]], and [[bilirubin]] are elevated. However, the following findings are typical for PSC:

* Autoantibodies ([[ANA]], [[ANNA]], [[pANCA]])
* Mildly increased [[AST]] and [[ALT]]

Symptoms of cholestasis often prompts morphological examination of bile ducts with [[MRCP]] (first choice) or [[ERCP]], which show multifocal strictures. Ultrasound can also be used to in the evaluation.

Biopsy and histology are not usually necessary for the diagnosis, unless there is suspicion of “small duct” PSC, which cannot be visualised on imaging.

It’s important to screen for concomitant IBD in patients diagnosed with PSC. It’s also important to exclude other causes of sclerosing cholangitis, including IgG4-associated cholangitis, bacterial infection and congenital anomalies.

== Treatment ==
There’s no treatment for PSC which slows the progression. [[UDCA]] is of unknown benefit for PSC, but many use it regardless, especially for cholestatic pruritus. Bile acid sequestrants like [[cholestyramine]] or [[rifampin]] may also help. [[ERCP]] with balloon dilation or stent insertion can be helpful in case of severe strictures.

Regular screening for complications is important. This includes:

* [[Cholangiocarcinoma]] and gallbladder cancer – annual [[ultrasound]]/[[MRCP]] and serum [[CA 19-9]]
* [[Colorectal cancer|Colon cancer]] (if concomitant UC) – [[colonoscopy]] every 1 – 5 years

[[Liver transplantation]] is the only definitive treatment but is reserved for end-stage disease or intractable pruritus.
[[Category:Gastroenterology]]