Igg4-Related Retroperitoneal Fibrosis Causing Ureter Stricture and Hydronephrosis: A Rare Case Report
Chi-Bo Lin, Hung-Jen Shih, Jesun Lin, Chang-Pao Chang, Bai-Fu Wang, Meng-Yi Yan, Sheng-Hsien Huang, Chun-Chi Chen, Herng-Jye Jiang, Jian-Xiang Zhang, Pao-Hwa Chen, Yi-Zhong Chen, Pan Yueh
Division of Pediatric Surgery, Department of Surgery, Changhua Christian Hospital
IgG4-related retroperitoneal fibrosis (RPF) is a rare disease characterized by sclerotic or fibrotic tissue in the peri-aortic or peri-iliac retroperitoneum that encases adjacent structures. This complication of IgG4-RPF can lead to ureteral obstruction, hydronephrosis and kidney injury. The frequency of hydronephrosis or ureter obstruction has been reported in up to two-thirds of patients with IgG4-related retroperitoneal fibrosis. We present a case of IgG4-related retroperitoneal fibrosis causing ureter stricture and hydronephrosis, including clinical presentation, images, and management.
A 51-year-old male patient presented with continuous left lower quadrant pain for days. We performed ureterorenoscopy due to left ureteral obstruction. Long-segment lower ureteral stricture was noted during the operation and only 4.7Fr ureteroscope could pass to middle ureter. Magnetic resonance imaging showed thickening of left bladder wall and infiltrative lesion in left retroperitoneal region, encasement of left middle and lower ureter. Computed tomography-guided biopsy was performed which disclosed chronic inflammation and fibrosis with increase of IgG4-plasma cells. We measured serum IgG4 levels which proved to be high. Therefore, diagnosing IgG4-related retroperitoneal fibrosis, we transferred the patient to division of Rheumatology. After corticosteroids administration, the patient responded favorably to the drug, with gradual regression of the lesion and symptoms. Currently, the patient is still following-up in the outpatient department.