IgG4-Related Disease (IgG4-RD) Autoimmune Pancreatitis (AIP) as an Initial Presentation of Systemic Lupus Erythematosus (SLE)

Abstract

Acute pancreatitis stemming from IgG4-Related Disease (IgG4-RD) seldom coincides with Systemic Lupus Erythematosus (SLE), highlighting the importance of investigating autoimmune conditions in patients with IgG4-RD. We present the case of a 57-year-old male with a medical history notable for hypertension, photosensitivity, arthritis, and malar rash, who presented with 6 weeks of persistent epigastric pain. Computed Tomography (CT) of the abdomen revealed hallmark features such as fat stranding around the pancreatic tail and gallbladder wall thickening, confirming the diagnosis of acute pancreatitis and cholecystitis. Post-cholecystectomy, histopathological examination of the gallbladder displayed IgG4-positive staining in multiple vessels, accompanied by perivascular inflammation and fibrinoid necrosis infiltrated by lymphocytes and neutrophils, confirming the diagnosis of IgG4-RD. Subsequent evaluation prompted by systemic manifestations revealed an ANA titer of 1:5120 and a dsDNA titer of 1:80, leading to the diagnosis of SLE. The patient later developed mononeuropathy, which improved upon initiation of immunosuppressive therapy. This case underscores the intricate interplay between IgG4-RD and SLE, an association documented to a limited extent in literature, thereby emphasizing the imperative of considering alternative autoimmune diseases with manifestations akin to IgG4-RD.