4th Year Advanced Endoscopy Fellows Program | October 2021-Hosmer

Hosmer_BIliary Stricture
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The patient is a 64-year-old male who was initially referred for a second opinion of jaundice. He had a three-month history of increasing back pain and presented with an episode of jaundice. His initial presentation showed cholestatic liver function tests (LFTs) and elevated lipase levels. On examination, he had scleral icterus and a non-tender, non-distended abdomen.<br /><br />The patient's past medical history included asthma/COPD, type 2 diabetes, hypertension, hyperlipidemia, and eczema. He had no prior surgeries and did not drink alcohol. His family history did not reveal any known gastrointestinal (GI) or liver disease.<br /><br />MRCP (magnetic resonance cholangiopancreatography) was conducted and suggested a distal biliary stricture with features of primary sclerosing cholangitis (PSC). The next steps in the workup included additional labs and an ERCP (endoscopic retrograde cholangiopancreatography).<br /><br />ERCP was performed, and a distal biliary stricture was identified. Brushings were obtained, and a stent was placed. However, there were no classic features of PSC, and the brushings were negative. The patient's CA 19-9 levels were elevated, and he developed cholangitis, which required a repeat ERCP for stent exchange. Despite these interventions, his liver function tests did not show improvement, so a liver biopsy was done, which did not provide evidence of PSC.<br /><br />Given the patient's history of pancreatitis, cholangitis, and distal common bile duct (CBD) stricture, along with elevated CA 19-9 levels, the differential diagnosis included IgG4 related disease. To confirm the diagnosis, additional tests were performed, including EUS (endoscopic ultrasound) with biopsies.<br /><br />The pathology and cytology results showed fibrosis and chronic inflammation in the biliary mucosa, with no significant IgG4 positive cells. The FNB (fine-needle biopsy) of the bile duct showed fibrosis with mild chronic inflammation and no significant IgG4 or IgG positive cells. The SpyBite biopsy of the bile duct showed mild active chronic inflammation and rare goblet cells, with an increase in IgG4 positive cells on stain. The FNB of the pancreatic head indicated fibrosis with lymphoplasmacytic infiltrate and increased IgG4 positive cells, suggestive of IgG4 related disease.<br /><br />Based on the workup, the patient's diagnosis was consistent with IgG4 related disease. Treatment with prednisone was initiated, and the patient was scheduled for repeat liver function tests and IgG4 levels after four weeks.
Keywords
jaundice
back pain
cholestatic liver function tests
lipase levels
scleral icterus
distal biliary stricture
primary sclerosing cholangitis
ERCP
CA 19-9 levels
IgG4 related disease