| 초록 |
Case Study: Retroperitoneal fibrosis (RPF) is a chronic fibroinflammatory disease that primarily involves the abdominal aorta and iliac arteries and may extend into the adjacent retroperitoneal space. RPF can lead to obstructive uropathy and subsequent renal failure. The etiology of RPF is heterogeneous, and anti-neutrophil cytoplasmic antibody (ANCA)–positive RPF is extremely rare. We report a case initially misdiagnosed as chronic kidney disease in which renal function recovered after appropriate treatment. A 76-year-old man presented with elevated serum creatinine (Cr) of 7.63 mg/dL and an estimated glomerular filtration rate (eGFR) of 6 mL/min/1.73 m² detected during a routine health examination without specific symptoms. His medical history included hypertension and benign prostatic hyperplasia. He had undergone surgery for sigmoid colon cancer 10 years earlier and had been declared cured five years prior. Laboratory tests at another hospital eight months before presentation showed Cr 2.05 mg/dL and eGFR 33.5 mL/min/1.73 m², suggesting progressive renal dysfunction initially presumed to represent chronic kidney disease. Abdominal CT performed five years earlier had shown no abnormalities. Renal ultrasonography revealed right hydronephrosis and atrophy of the left kidney. Non-contrast abdominal CT demonstrated a soft-tissue lesion surrounding the infrarenal abdominal aorta and both common iliac arteries, consistent with retroperitoneal fibrosis causing right kidney hydronephrosis (Figure 1). Serologic evaluation showed decreased complement levels, normal IgG4 levels, positive antinuclear antibody (ANA, 1:160), and positive P-ANCA (MPO antibody). No malignancy or other secondary causes of RPF were identified. Renal function improved after placement of a right ureteral double-J stent. Prednisolone 30 mg daily was initiated, and two months later renal function improved to Cr 1.69 mg/dL with eGFR 39 mL/min/1.73 m². This case highlights that ANCA-positive RPF may present without systemic vasculitis and cause acute kidney injury, emphasizing the importance of early diagnosis and treatment. |