Post written by Sooraj Tejaswi, MD, MSPH, FASGE, from the Division of Gastroenterology & Hepatology, University of California Davis School of Medicine, Sacramento, California.

This is a case series of 5 patients, demonstrating several unique and varied benign features of PSC. We describe a novel phenotypic classification of benign features, whereby we categorize findings into acute inflammation, chronic inflammation, and fibrostenosis. We demonstrate that this classification is dynamic, and the same patient can manifest features of different categories at different points in time. We also show that there can be synchronous overlap of features of different categories. For example, a patient can manifest both acute and chronic inflammation simultaneously and could go on to develop fibrostenosis.

We demonstrate that acute cholangitis can be seen in all 3 categories but is more commonly seen in patients with pigmented stones proximal to strictures. We describe an inflammatory mass that we categorize as 1 manifestation of acute inflammation and differentiate it from a malignant growth. We also differentiate the morphology of acute cholangitis from acute inflammation in this series. We demonstrate the morphology of benign dominant strictures and demonstrate the safety of cholangioscopic examination of dominant stricture after balloon dilation.

PSC is associated with a high risk of developing cholangiocarcinoma, especially in the presence of a dominant stricture. However, the majority of dominant strictures do not progress to cholangiocarcinoma. The diagnostic yield of the current method of fluoroscopic evaluation of dominant strictures and brush cytology remains suboptimal. While cholangioscopy has become a well-established modality in the diagnostic work up of cholangiocarcinoma, there is a scarcity of literature about the role of cholangioscopy in PSC using the newer generation of digital cholangioscopes that provide superior image quality and are easier to set up and maneuver. We present the first detailed video description of benign PSC, which will aid endoscopists in differentiating benign from malignant findings in PSC. This may lead to increased uptake of cholangioscopy in PSC to help tailor endoscopic management in PSC.

Based on currently available literature, predicting response to ERCP in PSC is difficult. We propose a novel phenotypic classification based on the benign cholangioscopic features of PSC. We found our classification to be helpful in selecting patients with fibrostenotic disease who would benefit from endoscopic therapy such as stricture dilation, while reducing endoscopic interventions in patients with acute inflammatory strictures. We also hypothesize that our classification could help with predicting the long-term prognosis of PSC, and we are currently studying this.

Based on our experience, cholangioscopy can be performed safely and effectively in PSC, even in the presence of dominant strictures. We have proposed a step-by-step guide for undertaking cholangioscopy in PSC.

We propose that cholangioscopy-aided phenotypic categorization of benign PSC into acute inflammation, chronic inflammation, and fibrostenosis can help tailor endoscopic management. For example, a patient with fibrostenotic disease and/or dominant stricture in the absence of malignancy, is more likely to respond to balloon dilation. However, acute inflammation, which can also manifest as a stricture on non-invasive cross-sectional imaging and fluoroscopy, is unlikely to respond to balloon dilation. Such patients may benefit from early liver transplant evaluation if they are experiencing ongoing pruritus, fatigue, cholestasis, or recurrent acute cholangitis.

Pigmented stones proximal to strictures can be missed on cross-sectional imaging, and even on fluoroscopy, and can be the nidus of recurrent acute cholangitis. Cholangioscopy helps in both identification and clearance of such stones with the aid of electrohydraulic lithotripsy.

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