Obstructive jaundice caused by IgG4-related disease with multiorgan involvement.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with multiorgan involvement. The variable clinical manifestations and image findings of this disease often present a diagnostic dilemma for physicians. Herein, we describe a patient with IgG4-RD involving the kidney, retroperitoneum, pancreas, and biliary tracts, successfully treated with steroids. A 68-year-old man presented to our emergency room with a 2-week history of jaundice, tea-color urine, and poor appetite. He denied presence of fever, dyspnea, abdominal pain, or dysuria recently. Relevant laboratory data showed elevated liver enzymes and bilirubinemia (SGOT = 82 IU/L, SGPT = 174 IU/L, ALP = 196 IU/L, GGT = 340 IU/L, total bilirubin = 12 mg/dl, direct bilirubin = 7.51 mg/dl) with normal C-Reactive protein (CRP), blood cell counts and renal function. Abdominal computed tomography (CT) showed enhancing soft tissue lesions at bilateral renal pelvises (Figure 1A, arrow) and paraaortic regions (Figure 1B, arrow), diffuse swelling of the pancreas with a peripheral poorly enhanced halo (Figure C, short arrow) and dilatation of intra-hepatic and extra-hepatic bile ducts associated with enhancing wall thickenings (Figure 1 D, arrow). Urine cytology, performed twice, was negative for malignant cells. Ureterorenoscopy