3 pictures!

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease characterized by idiopathic intrahepatic and extrahepatic bile duct inflammation and fibrosis leading to multifocal bile duct strictures.
The main clinical manifestation of autoimmune liver disease
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Because PSC is a rare disease, the clinical diagnosis of PSC is challenging and requires the acuity of the physician's diagnosis
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Next, let's take a look at the interpretation of the latest guidelines for PSC and how to improve the diagnosis rate of PSC
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1 The pathogenesis of PSC The pathogenesis of PSC is still unclear, and it is currently believed to be caused by the joint participation of multiple factors such as genetics, environment, immunity, bile acid metabolism and intestinal flora
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According to the site of bile duct damage, PSC can be divided into large duct type, small duct type, and full duct type (Figure 1)
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Figure 1∣ Pathogenesis and classification of PSC 2.
The diagnosis of PSC has various clinical manifestations, and most of them are asymptomatic in the early stage.
Some patients are diagnosed with PSC during physical examination or liver function screening due to inflammatory bowel disease
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Therefore, when PSC is suspected, the most useful test is magnetic resonance cholangiopancreatography (MRCP), along with serum AMA and IgG4 testing to rule out secondary biliary cholangitis and IgG4-related sclerosing cholangitis; Liver biopsy may be performed in patients with ambiguous, suspected small duct-type PSC, or suspected overlapping other diseases, and liver biopsy is not recommended as a routine diagnostic tool for PSC (Figure 2)
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Figure 2 ∣ Diagnosis of PSC 3 Treatment of PSC For the treatment of PSC, there is still a lack of specific drugs, and the recommended treatment is ursodeoxycholic acid (UDCA) 15 mg/kg/d
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Glucocorticoids are not routinely prescribed in patients with PSC, and can only be used in patients with overlapping autoimmune hepatitis (AIH) or PSC with features of AIH
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Due to the lack of effective treatment options, clinical treatment is still based on symptomatic treatment.

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For PSC patients with severe itching, drugs such as sertraline, rifampicin, naltrexone or cholestyramine can be used
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In the event of overt stenosis of the bile duct, endoscopic balloon dilation or short-term stent placement for bile duct drainage can be performed (Figure 3)
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Figure 3 ∣ Treatment of PSC Liver transplantation is the only effective treatment for PSC.
Liver transplantation may be considered for patients with reduced quality of life and end-stage liver disease, but the recurrence of disease should be closely monitored after liver transplantation
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References: [1] Liver Disease Branch of Chinese Medical Association.
Guidelines for the diagnosis and treatment of primary biliary cholangitis (2021) [J].
Journal of Clinical Hepatobiliary Diseases, 2022, 38(1): 35-41.
[2 ]Andrea A.
et al.
Primary Sclerosing Cholangitis: What the Gastroenterologist and Hepatologist Needs to Know.
Clin Liver Dis 2017 Nov;21(4):725-737.