Immunoglobulin G4-related Disease of the Genitourinary System: Spectrum of Imaging Findings and Clinical Pathologic Features

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by focal or diffuse organ infiltration of IgG4-bearing plasma cells. The diagnosis of IgG4-RD is based on a combination of clinical, serologic, radiologic, and histopathologic findings. IgG4-RD has been reported to affect almost all organ systems. The kidney is the most frequently involved of the genitourinary organs. The most common renal manifestation of IgG4-RD is IgG4-RD tubulointerstitial nephritis, followed by membranous glomerulonephropathy and, less frequently, obstructive nephropathy involving the renal pelvis, ureter, or retroperitoneum. Renal parenchymal lesions may appear as multiple nodular lesions, diffuse patchy infiltrative lesions, or a single nodular lesion. Multiple small nodular cortical lesions are the most common imaging findings of IgG4-RD involving the kidney. Renal pelvic, sinus, or perinephric lesions can also occur. IgG4-RD involvement of other genitourinary organs including the ureter, bladder, urethra, and male and female reproductive organs is rare compared with kidney involvement but may show variable imaging findings such as a localized mass within or surrounding the involved organ or diffuse enlargement of the involved organ. Imaging findings of IgG4-RD involving the genitourinary system are nonspecific but should be differentiated from inflammatory and neoplastic lesions that mimic IgG4-RD. (C) RSNA, 2020