SHM Converge Scientific Abstract ePoster Gallery-Multisystem Igg4-Related Disease Presenting With Recurrent Pancreatitis, Biliary Stricture, and Submandibular Lymphadenopathy

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This case describes a 43-year-old Hispanic man with recurrent pancreatitis, biliary obstruction, submandibular swelling, and left eye proptosis with clear drainage. He initially had a history of gallstone pancreatitis treated with cholecystectomy and CBD stent placement, but later returned with abdominal pain, nausea, vomiting, and poor oral intake. Labs showed marked pancreatitis and cholestatic liver injury. Imaging revealed acute pancreatitis, intrahepatic bile duct dilation, and an occluded CBD stent, which was removed by ERCP with symptomatic improvement.<br /><br />Because of persistent multisystem findings, additional workup was pursued. He had an elevated globulin gap, markedly high serum IgG4, and peripheral eosinophilia. Clinical suspicion for IgG4-related disease increased due to the combination of recurrent pancreatitis, biliary stricture, submandibular and mesenteric lymphadenopathy, and orbital/lacrimal gland involvement. A mandibular gland biopsy demonstrated dense lymphoplasmacytic infiltration with eosinophils and storiform fibrosis, confirming IgG4-related sialadenitis. Infectious testing, including hepatitis panel and TB testing, was negative.<br /><br />He was treated with high-dose corticosteroids followed by rituximab because of multisystem involvement. After treatment, he improved significantly, with resolution of proptosis and eye drainage, decreased lymph node enlargement, and better biliary and pancreatic function.<br /><br />The key lesson is that IgG4-related disease can mimic malignancy or infection and present in multiple organs, especially the pancreas, biliary tree, salivary glands, and lacrimal glands. Diagnosis depends on elevated IgG4, supportive imaging, and confirmatory tissue biopsy. Early corticosteroid therapy is highly effective and can prevent permanent organ damage.

Asset Subtitle

Mahmuda Islam

Meta Tag

Author List Aniqa Islam, Mahmuda Islam

Category Clinical Vignettes

Concept IgG4-Related Disease

Concept Serum IgG4

Concept Lymphoplasmacytic infiltrate

Concept Storiform fibrosis

Concept Corticosteroid

Distinguished Non-Finalist

Presenter Organization Utsouthwestern Medical Center

Presenting Author Mahmuda Islam

Track Adult

Keywords

IgG4-related disease

recurrent pancreatitis

biliary obstruction

submandibular swelling

left eye proptosis

serum IgG4

lymphoplasmacytic infiltration

storiform fibrosis

rituximab therapy

corticosteroid treatment

IgG4-Related Disease

Serum IgG4

Lymphoplasmacytic infiltrate

Storiform fibrosis

Corticosteroid