Immunoglobulin G4–Related Disease of the Genitourinary System

BACKGROUND: Immunoglobulin G4–related disease (IgG4-RD) is an immune-mediated disease that is often misdiagnosed given its rare incidence and misleading presentation. Prior case studies have described IgG4-RD involving the ovaries, uterus, and bladder. This article describes the first case of vaginal IgG4-RD. CASE: A 76-year-old woman presented with episodes of vaginal spotting, vaginal cuff friability, and benign inflammation on vaginal pathology. She was unsuccessfully treated with topical estrogen and antibiotics. She ultimately was found to have a progressive, obstructive pelvic mass near the vaginal cuff that was surgically excised, with mass pathology showing lymphoplasmacytic disease. The mass recurred and repeat sampling of the vaginal apex confirmed IgG4-RD. CONCLUSION: This report showcases the novel finding of vaginal IgG4-RD. The patient's decade-long course before diagnosis highlights the challenges in identifying this disease. This case report aims to increase awareness of vaginal IgG4-RD, leading to timely diagnosis and treatment.