Urinary bladder involvement in IgG4-related disease: A case-based review

Abstract

ABSTRACT Immunoglobulin G4–related disease (IgG4-RD) is an immune-driven fibroinflammatory disease that presents as tumefactive lesions that not only commonly affects the pancreas, lacrimal and salivary glands, lung, liver and kidney but can also affect any organs. However, involvement of the urinary bladder in IgG4-RD is rarely reported. We describe a case of IgG4-RD involving the urinary bladder mimicking carcinoma and review the published literature—a 39-year-old male presented with complaints of dysuria, urgency and hesitancy. Ultrasound revealed a hyperechoic lesion protruding from the anterior of the urinary bladder wall with partial obstruction to bladder outflow, likely to be a pedunculated bladder mass with high suspicion for malignancy. A contrast-enhanced computed tomography abdomen showed a large irregular lobulated heterogeneously enhancing lesion involving the anteroinferior wall of the urinary bladder extending from mid-body up to the neck region with significant perivesical fat stranding and multiple ill-defined perivesical deposits along with hypodense soft tissue lesion in the perigastric region at the level of the body of the stomach. CT-guided perigastric and ultrasound-guided biopsy from the urinary bladder mass confirmed the diagnosis of IgG4-RD. The patient was treated with glucocorticoids. He is doing well after a 1-year follow-up without recurrence, and a repeat ultrasound showed a significant reduction in the size of the urinary bladder mass. The diagnosis of IgG4-RD should be considered in the differential diagnosis of a urinary bladder mass. High index of suspicion and prompt initiation of therapy are required to minimise residual damage and the need for surgical intervention.