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

Abstract

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