Primary sclerosing cholangitis: Difference between revisions
(Created page with "'''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 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...") |
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'''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 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. | '''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 == | == 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. | 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. | Because most patients with PSC also have concomitant IBD, symptoms of IBD can also be present. | ||
== Diagnosis and evaluation == | == Diagnosis and evaluation == | ||
As with any cholestasis, ALP, GGT, and bilirubin are elevated. However, the following findings are typical for PSC: | As with any [[cholestasis]], [[ALP]], [[GGT]], and [[bilirubin]] are elevated. However, the following findings are typical for PSC: | ||
* Autoantibodies (ANA, ANNA, pANCA) | * Autoantibodies ([[ANA]], [[ANNA]], [[pANCA]]) | ||
* Mildly increased AST and ALT | * 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. | 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. | Biopsy and histology are not usually necessary for the diagnosis, unless there is suspicion of “small duct” PSC, which cannot be visualised on imaging. | ||
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== Treatment == | == 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. | 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: | Regular screening for complications is important. This includes: | ||
* Cholangiocarcinoma and gallbladder cancer – annual ultrasound/MRCP and serum CA 19-9 | * [[Cholangiocarcinoma]] and gallbladder cancer – annual [[ultrasound]]/[[MRCP]] and serum [[CA 19-9]] | ||
* Colon cancer (if concomitant UC) – colonoscopy every 1 – 5 years | * [[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. | [[Liver transplantation]] is the only definitive treatment but is reserved for end-stage disease or intractable pruritus. | ||
[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
Latest revision as of 13:23, 30 October 2023
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 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:
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
- 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.