IgG4-Related Sclerosing Cholangitis Without Elevated Serum IgG4 Levels

Abstract

IgG4 related sclerosing cholangitis is known for elevated IgG4 levels and dense infiltration of IgG4 positive plasma cells with fibrosis of bile duct. Multiple biliary stricture episodes, especially in those with a history of pancreatitis, should warrant further workup. We report a case of a man who despite having a negative biopsy on ERCP and normal IgG4 levels was diagnosed with IgG4 related sclerosing cholangitis. A 68 year-old male presented with abdominal pain for five days and was told by PCP to go to the ED due to abnormal liver tests. He had a history of pancreatitis, biliary stricture with stent placement, HIV on HAART, and was treated for Hepatitis C. Vital signs were within normal limits. Physical exam was significant for jaundice, scleral icterus, and right upper quadrant abdominal tenderness. Lab results displayed an INR peaking at 1.8 and Tbili/Dbili/ALT/AST/ALP of 9.8/5.2/122/65/299, respectively. Hepatitis serologies showed adequate Hepatitis B immunization and undetectable Hepatitis C viral load. HIV viral load was undetectable. MRCP showed hilar mass affecting common bile and right hepatic ducts thought to be a Klatskin tumor. ERCP completed, stent placed, and hilar mass biopsied but found to be unrevealing. Outpatient follow up given presumed Klatskin tumor and patient with continued symptoms yielded left hepatectomy, caudate lobe and common bile duct resection with Roux-en-y hepaticojejunostomy. Pathology showed IgG4 related sclerosing cholangitis. IgG levels elevated at 2596, but IgG4 levels were toward the upper limit of normal at 83.9. Patient started on prednisone for treatment with improvement of symptoms. Distinctions between sclerosing cholangitis subtypes are critical due to prognosis differences. The utility of an ERCP performed biopsy may be low yield given they are often of limited depth. There are not well known diagnostic criteria. If biopsy results and serum levels of IgG4 are positive or patient responds to known treatment of prednisone, then diagnosis can be established. It is important to keep a broad differential when a patient presents with abdominal pain and liver tests significant for biliary obstruction. In this case, given our patient was with continued symptoms, IgG4 related sclerosing cholangitis should not be thrown to the wayside until all diagnostic methods have been utilized.Figure: Image 1: Hilar Mass compatible with presumed Klatskin tumor.