Recurrent and Extensive Idiopathic Granulomatous Ureteritis: A Localized Hyperimmune Disease with Genetic Predisposition

Idiopathic granulomatous ureteritis (IGU) is a rare autoimmune disorder. Multiple case reports led to defining its clinicopathological inclusion criteria in 1997. Surgical resection and primary reanastomosis, of such pseudotumor, were considered definitive management 4-months corticosteroid-therapy was used once for persistent ureteric lesion despite 3-months stenting. Long-term follow-up disease limited extensive recurrent lacking. In our report, 47-year-man had history biopsy-proven IGU 4 years ago that treated with ureteral reimplantation cystoplastic (augmented) bladder. Moreover, he received Corticosteroids Azathioprine total 2 avoid recurrence. Two later, presented abdominal pains, urinary tract infections ultimately; bladder neck disease. Cystoscopic examination revealed masses severe left stricture. Biopsy the lesions confirmed idiopathic He improved, immunosuppressive therapy included 3 months Mycophenolate mofetil followed by maintenance mofetil. Previous animal studies have shown local hyperimmune response malformation transitional epithelium genetically predisposed mice indicating genetic predisposition immune-mediated expression. Hence, patient, we proposed long-term follow-up. conclusion; report confirms etiology disorder provides new line variant.