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Abstract titles should be brief and reflect the content of the abstract.
IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis, affecting multiple organs. Retroperitoneal fibrosis (RPF) is a recognized manifestation that may cause ureteral obstruction and chronic kidney disease (CKD) if diagnosis or intervention is delayed. Isolated urinary tract involvement is rare and often underreported, and diagnostic delay can lead to irreversible renal damage despite appropriate therapy.
We report the case of a 49-year-old male with IgG4-related RPF, stage 3a CKD, bilateral hydronephrosis, and exclusive right renal atrophy. Clinical, laboratory, and imaging data were analyzed, including computed tomography (CT), renal scintigraphy (DMSA), and histopathology. Laboratory results showed serum creatinine 1.8 mg/dL, creatinine clearance 46 mL/min/1.73 m², and elevated IgG4 (124 mg/dL). Urinalysis revealed marked hematuria and leukocyturia (>1,000,000/mL). CT demonstrated a retroperitoneal mass compressing both ureters, causing bilateral hydronephrosis and right renal atrophy. DMSA scintigraphy revealed right renal function of 8% and left of 92%.
The patient initially presented in 2021 with a retroperitoneal mass but no urological intervention, leading to progressive renal decline. In 2023, he was hospitalized with uremic symptoms requiring renal replacement therapy and bilateral double-J stent placement. Immunosuppressive therapy with prednisolone (10 mg/day) and mycophenolate mofetil (360 mg/day) produced partial improvement. Despite treatment, renal function recovery was incomplete due to advanced fibrosis. Recurrent urinary infections with Pseudomonas aeruginosa were managed with antibiotics.
A literature review of 60 reported IgG4-related RPF cases showed that corticosteroid therapy was used in 75% (45/60) of patients, while immunomodulatory agents (rituximab, mycophenolate, azathioprine) were less common. Hemodialysis was required in 10% (6/60) due to advanced renal impairment. Urological decompression procedures included double-J stents in 5% (3/60), with an additional 3–4 patients described with unspecified ureteral stents; nephrostomy was performed in 10% (6/60), and nephrectomy in 7% (4/60). Unilateral renal atrophy was reported in only 1 of 60 cases (≈1.7%), underscoring the rarity and severity of the present presentation.
This case illustrates an uncommon presentation of IgG4-related RPF restricted to the urinary system, resulting in complete unilateral renal atrophy. The prolonged diagnostic delay (2 years) contributed to irreversible fibrosis and only partial response to immunosuppressive therapy. IgG4-RD should be considered in patients with RPF or unexplained ureteral obstruction to enable early diagnosis, decompression, and timely immunosuppression. Therefore, prompt recognition is crucial to prevent irreversible renal injury and preserve function.
