| Primary sclerosing cholangitis | |
|---|---|
| Other names | Stenosing cholangitis, fibrosing cholangitis, chronic obliterative cholangitis |
 | |
| Cholangiogram of primary sclerosing cholangitis. | |
| Specialty | Gastroenterology |
| Symptoms | Yellow discoloration, itching, right upper abdominal pain[1] |
| Complications | Liver failure, gallbladder cancer, bile duct cancer, bile duct infection[2] |
| Usual onset | 30 to 50 years old[2] |
| Duration | Long-term[2] |
| Types | Classic, small-duct, associated with autoimmune hepatitis[1] |
| Causes | Unknown[1] |
| Risk factors | Inflammatory bowel disease, family history[2] |
| Diagnostic method | Elevated alkaline phosphatase, MRCP, ERCP, liver biopsy[1] |
| Differential diagnosis | Autoimmune hepatitis, pancreatitis, primary biliary cirrhosis, common bile duct stones[1] |
| Treatment | Immunosuppressants, cholestyramine, liver transplant[1] |
| Prognosis | Life expectancy 10 years without transplant[1] |
| Frequency | 11 per 100,000 people[2] |
Primary sclerosing cholangitis (PSC) is a long-term disease in which the bile ducts inside and outside the liver become inflamed and scarred, and are eventually narrowed or blocked.[2] Many people initially have no symptoms; however over time may develop liver disease with yellow discoloration of the skin and eyes, itching, and right upper abdominal pain.[1] Complications may include liver failure, gallbladder cancer, bile duct cancer, bile duct infection, and fat soluble vitamin deficiencies.[2][1]
The cause is unknown.[1] In approximately 70% of cases the person has inflammatory bowel disease (IBD), most often ulcerative colitis (UC) and conversely about 7.5% of people with UC have PSC.[2][1] Other risk factors include a family history of the disease and being a nonsmoker.[1] The underlying mechanism is believed to be that of an autoimmune disease.[2] Diagnosis may be made based a persistently elevated alkaline phosphatase and MRCP after ruling out other possible causes.[1]
There is no cure.[1] Efforts to slow the disease may include the use of immunosuppressants.[1] Cholestyramine or naltrexone may be used to improve itchiness.[1] A liver transplant is eventually required in about 40% of people, and despite transplantation the disease recurs in 25% of cases.[3] Without a liver transplant the typical life expectancy is 10 years.[1]
PSC is a rare, affecting about 11 per 100,000 people.[2] Onset is usually in peoples 30s or 40s.[2] People of Northern European ancestry are more commonly affected than those of Asian descent. Males are affected twice as often as females.[2][1]The disease was first described in the mid-1800s but was not fully characterized until the 1970s.[4]
References edit
- ^ a b c d e f g h i j k l m n o p q r Rawla, P; Samant, H (January 2020). "Primary Sclerosing Cholangitis". PMID 30725866.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d e f g h i j k l "Definition & Facts for Primary Sclerosing Cholangitis | NIDDK". National Institute of Diabetes and Digestive and Kidney Diseases. Retrieved 11 February 2021.
- ^ Lazaridis, KN; LaRusso, NF (September 2016). "Primary Sclerosing Cholangitis". New England Journal of Medicine (Review). 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMC 5553912. PMID 27653566.
- ^ Williamson, KD; Chapman, RW (June 2015). "Primary sclerosing cholangitis: a clinical update". British Medical Bulletin. 114 (1): 53–64. doi:10.1093/bmb/ldv019. PMID 25981516.