Secondary Sclerosing Cholangitis: Pathogenesis, Diagnosis, and Management

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Key points

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    Secondary sclerosing cholangitis (SSC) is a rare disease entity with complex pathogenesis.

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    Common causes for SSC include obstruction, infection, ischemia, and critical illness.

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    Although initially asymptomatic, patients with SSC may present later with pruritus, abdominal pain, and jaundice.

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    The cholangiographic finding of isolated peripheral ductal abnormalities suggests SSC over primary sclerosing cholangitis.

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    Management should address the underlying cause of SSC, and liver transplantation is an

Common causes

Unlike PSC, the origin of which is still under investigation, common causes for SSC are evident and can be treated accordingly. Common causes include obstruction, ischemic insult, infections, and immunologic disease. Imaging and diagnostic modalities can help differentiate secondary causes, and can hence aid in implementing the appropriate treatment in a timely and effective manner. Table 1 provides a summary of common causes and their related pathogenesis.

Clinical symptoms and diagnosis

Most patients at the initial stages of the disease are asymptomatic, with elevated alkaline phosphatase and gamma glutamyltransferase levels. Several symptoms may occur as the disease progresses, which may include pruritus, abdominal pain, and jaundice. In patients with SSC, recurrent episodes of bacterial cholangitis from ascending infection are common.48, 49, 50

After the identification of abnormal liver test results, patients should undergo ultrasonography to detect biliary abnormalities

Radiologic differentiation

Cholangiographic findings may be helpful in distinguishing SSC from PSC. Diffuse ductal narrowing and multifocal strictures suggest PSC, whereas isolated peripheral ductal abnormalities suggest SSC. Recurrent pyogenic cholangitis may show sudden ductal cutoff, intrahepatic bile collections, and ductal stones. Autoimmune pancreatitis may show changes to the pancreatic duct, whereas AIDS cholangiopathy is characterized by papillary stenosis and accompanying intrahepatic disease.

Management, complications, and outcomes

The management of SSC depends on the underlying cause. Patients with recurrent pyogenic cholangitis may benefit from prompt supportive care and institution of empiric antibiotics, followed by monitoring. If decompensation occurs or the disease is persistent, surgical intervention may be necessary. Surgery focuses on drainage and exploration of the bile ducts. Endoscopic intervention may also be used when needed, and long-term drainage may be the only option in a few patients with widespread

Summary

Because of the reversible nature of secondary sclerosing cholangitis, a high suspicion for the diagnosis should be maintained, especially in patients with PSC with unclear diagnostic features.

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