Westchester Medical Center Bronx, NY, United States
Aaron Weiss, MD, Cynthia Cohen, MD, Michael Rosman, MD, Virendra Tewari, MD Westchester Medical Center, Valhalla, NY
Introduction: An immune-mediated fibroinflammatory condition, IgG4 related disease can affect multiple organs. Biliary involvement leads to IgG4-Scleorising Cholangitis (IgG4-SC) with extrahepatic biliary strictures, intrahepatic biliary strictures or both. Disease evolution from one pattern to another is not described. We discuss an interesting case wherein a patient initially had only extrahepatic bile duct involvement but subsequently developed intrahepatic biliary disease.
Case Description/Methods: A 60-year-old man was transferred to evaluate an incidental finding on CT scan: diffuse bulbous appearance of pancreas with a possible pancreatic mass and marked biliary dilation. He had mild abdominal pain, new onset diabetes and elevated liver tests: ALT 401, ALP 599 and TB 1.8. He underwent EUS-FNA which was negative for malignancy. ERCP showed a distal CBD stricture, and a plastic stent was placed. IgG4 level at this time was normal. He was started on prednisone and follow-up labs were markedly improved. However, at his next outpatient GI appointment, he was found to have abruptly stopped glucocorticoids without a taper, and his repeat labs showed elevated liver tests, and a very high CA 19-9. Due to concern for malignancy, he underwent another ERCP which demonstrated resolution of the distal stricture but noted a new proximal extrahepatic stricture, which was dilated with a covered metal stent. Biliary brushings were negative for malignancy. He did not follow our recommendation to resume steroids. Labs now showed a positive IgG4 and still-high CA 19-9 and LFTs. ERCP with cholangioscopy was then done, and at this time the extrahepatic strictures had resolved but new intrahepatic biliary strictures were present. Biopsies/brushings were again negative. Two long plastic stents were placed in bilateral lobes, and prednisone with azathioprine was started. Follow-up labs showed normalization of liver tests, CA 19-9, and IgG4 and he is currently asymptomatic.
Discussion: There are several interesting aspects to our case. Firstly, after a thorough initial work-up and exclusion of malignancy, he was started on steroids despite normal IgG4 based on the imaging and overall clinical picture. Interestingly, his IgG4 only became positive several months into the disease course. Additionally, his disease phenotype evolved from extrahepatic biliary to intrahepatic biliary involvement. Finally, this case emphasizes the role of immunosuppression in achieving complete resolution in spite of a very concerning presentation.
Figure: A) ERCP showing distal intrahepatic bile duct stricture. B) ERCP showing proximal intrahepatic bile duct stricture. C) ERCP showing extrahepatic bile duct stricture. D) Graph showing the trend in lab values in association with stenting and immunosuppression. Legend: Blue- ALT; Orange- ALP; Gray- CA 19-9; Yellow- IgG4. The patient received an initial stent and prednisone in Dec 2019 followed by improvement in lab values. After discontinuation of therapy in Feb 2020, he had a marked increase in liver tests, newly elevated IgG4 and CA 19-9 levels and a new intrahepatic biliary stricture in July 2020. With subsequent stenting and reinitiating of immunosuppression, all his lab values again improved.
Disclosures: Aaron Weiss indicated no relevant financial relationships. Cynthia Cohen indicated no relevant financial relationships. Michael Rosman indicated no relevant financial relationships. Virendra Tewari indicated no relevant financial relationships.
Aaron Weiss, MD, Cynthia Cohen, MD, Michael Rosman, MD, Virendra Tewari, MD. P2202 - A Case of IgG4-Related Sclerosing Cholangitis With Biliary Involvement Progressing Proximally Following Corticosteroid Withdrawal, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.