• Larabe Farrukh, Muhammad Farhan Akhtar, Hafiza Hareem Waqar, and Ruben Peredo-Wende.
• Department of Medicine, Albany Medical Center, Albany, NY, USA. Electronic address: Larabefarrukh123@gmail.com.
• Am. J. Med. Sci. 2024 Jan 1; 367 (1): 677167-71.

AbstractIgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterized by dense infiltration of IgG4-positive plasma cells in the affected tissue along with characteristic storiform fibrosis that can lead to the development of tumefactive lesions in any organ. CA19-9 is a marker for pancreato-biliary malignancy, however mild to moderate elevation of CA 19-9 can also be observed in IgG4-RD autoimmune pancreatitis (AIP) and sclerosing cholangitis (IgG4-SC). Therefore, it becomes difficult to differentiate between these entities. We describe the case of a 65-year-old male with history of IgG4-RD, presenting with jaundice and abdominal pain. He was found to have a pancreatic mass with significantly elevated IgG4 162 (2-96 mg/dL and CA19-9 levels 2830 (0-35 U/ml). Patient underwent ERCP and biopsy, which ruled out pancreatic cancer and cholangiocarcinoma. He was diagnosed with IgG4-RD autoimmune pancreatitis (AIP) and sclerosing cholangitis. Treatment with steroids and rituximab resulted in significant improvement in the bilirubin and a dramatic decrease in CA19-9 levels.Copyright © 2023 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

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