SHM Converge Scientific Abstract ePoster Gallery-Looking for a Needle in the Haystack

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A 73-year-old man with diabetes and hypothyroidism presented with 10 days of dark urine, pale stools, progressive jaundice, pruritus, and mild weight loss. He was hemodynamically stable but markedly jaundiced on exam. Labs showed a cholestatic and inflammatory pattern with elevated bilirubin, alkaline phosphatase, transaminases, lipase, and CA 19-9.<br /><br />CT abdomen/pelvis raised strong concern for malignancy, showing a 2.4 cm right renal mass, 2.6 cm peripancreatic lymphadenopathy causing biliary obstruction, and a 7.6 cm presacral soft tissue mass. ERCP with biliary stenting revealed mass-like thickening of the intrapancreatic bile duct, again suggestive of bile duct neoplasm, so endoscopic ultrasound with FNA was performed. The patient improved dramatically after stenting, with resolution of symptoms and hyperbilirubinemia.<br /><br />However, pathology from the bile duct FNA showed only bile duct epithelial cells and inflammatory cells, without dysplasia. Lymph node biopsy was also negative for malignancy, showing only inflammatory cells. Repeat FNA remained negative, prompting a broader differential diagnosis and testing for IgG4-related disease. Serum IgG4 was markedly elevated at 492 mg/dL (normal 0–123 mg/dL).<br /><br />This case highlights IgG4-related disease as a fibroinflammatory, immune-mediated condition that can mimic cancer by producing tumor-like masses in multiple organs. Common manifestations include autoimmune pancreatitis, sclerosing cholangitis, salivary gland disease, orbital disease, and retroperitoneal fibrosis. Because it can present with obstructive jaundice, elevated CA 19-9, and imaging findings suspicious for malignancy, it should be considered when initial cancer workup is negative.<br /><br />The main lessons are to maintain suspicion for IgG4-related disease in patients with multi-organ masses and negative biopsies, and to recognize that treatment is typically multimodal, often involving steroids, immunosuppression, and procedural interventions for obstruction.

Asset Subtitle

Vineet Gupta

Meta Tag

Author List Faizah Shareef, Farid Ghamsari, Shawn Khrishnan, Vineet Gupta

Category Clinical Vignettes

Concept IgG4-Related Disease

Concept Corticosteroid

Concept Autoimmune Pancreatitis

Concept Primary Sclerosing Cholangitis

Concept Tumor-like mass

Distinguished Non-Finalist

Presenter Organization University of California, San Diego (UCSD)

Presenting Author Vineet Gupta

Track Adult

Keywords

IgG4-related disease

obstructive jaundice

bile duct mass

autoimmune pancreatitis

sclerosing cholangitis

CA 19-9

biliary obstruction

peripancreatic lymphadenopathy

retroperitoneal fibrosis

endoscopic ultrasound FNA

IgG4-Related Disease

Corticosteroid

Autoimmune Pancreatitis

Primary Sclerosing Cholangitis

Tumor-like mass