Primary Sclerosing Cholangitis: Statement Analysis
Statement (b) is correct—a similar cholangiographic picture can be seen in parasitic cholangitis infections in AIDS patients, and statement (c) is correct—PSC typically manifests in middle age. 1, 2
Detailed Analysis of Each Statement
Statement (a): "Affects only the intrahepatic bile ducts" - FALSE
PSC characteristically affects both intrahepatic AND extrahepatic bile ducts together in most cases. 1
- PSC is defined as inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts, leading to multifocal strictures and segmental dilatations 1
- While isolated involvement of only intrahepatic or only extrahepatic ducts can occasionally occur, this is not the typical pattern 3
- The disease produces a characteristic "beaded" appearance on cholangiography due to alternating strictures and dilatations affecting the entire biliary tree 3
Statement (b): "Similar picture in parasitic cholangitis infections in AIDS patients" - TRUE
Secondary sclerosing cholangitis from AIDS cholangiopathy can mimic PSC cholangiographically. 1
- AIDS cholangiopathy is explicitly listed as a secondary cause of sclerosing cholangitis that must be excluded before diagnosing PSC 1, 4
- Multiple conditions can produce cholangiographic findings similar to PSC, requiring careful exclusion of secondary causes 1
- The clinical history and presence/absence of inflammatory bowel disease help distinguish primary from secondary sclerosing cholangitis 1
Statement (c): "The disease manifests in middle age" - TRUE
PSC typically presents in young to middle-aged adults, with a predominance in males. 5
- The disease affects all ages but shows predominance in young males 5
- Many patients are asymptomatic at diagnosis, with disease identified incidentally during screening or evaluation of cholestatic liver enzymes 1, 2
- Approximately 60-80% have concomitant inflammatory bowel disease at presentation 1
Statement (d): "The initial diagnostic approach is ERCP" - FALSE
Magnetic resonance cholangiopancreatography (MRCP) is the first-line diagnostic modality for PSC, NOT ERCP. 6, 4
- MRCP has sensitivity of 80-100% and specificity of 89-100% for diagnosing PSC 6
- ERCP should only be performed after expert multidisciplinary assessment to justify endoscopic intervention 1, 4
- ERCP is reserved for patients requiring tissue acquisition (to exclude cholangiocarcinoma) or therapeutic intervention such as dominant stricture dilatation 6, 4
- When ERCP is performed, prophylactic antibiotics are mandatory 1, 4
- The British Society of Gastroenterology strongly recommends that patients with PSC should not undergo ERCP until expert multidisciplinary assessment justifies intervention 1
Common pitfall: Using ERCP as initial diagnostic test exposes patients to unnecessary procedural risks (pancreatitis, cholangitis, perforation) when non-invasive MRCP provides excellent diagnostic accuracy 6, 4
Statement (e): "Often manifests with episodes of cholangitis" - FALSE
Episodes of bacterial cholangitis (fever and chills) are very uncommon at initial presentation in PSC. 1
- Cholangitis is very uncommon at presentation in the absence of prior biliary surgery or instrumentation such as ERCP 1
- Typical presenting symptoms include right upper quadrant discomfort, fatigue, pruritus, and weight loss—not cholangitis 1, 2
- Many patients are completely asymptomatic at diagnosis, identified only through incidentally elevated alkaline phosphatase 1, 2
- Bacterial cholangitis may occur later in disease course at sites of dominant strictures, but this is not a typical presenting feature 2
Clinical caveat: If a patient presents with recurrent cholangitis episodes, consider secondary causes of sclerosing cholangitis such as choledocholithiasis or recurrent pyogenic cholangitis rather than classic PSC 1, 4