Xanthogranulomatous pyelonephritis complicating sepsis and abdominal compartment syndrome.

Introduction and Importance: Xanthogranulomatous pyelonephritis (XGP) is a rare chronic kidney infection characterized by tissue destruction and lipid-laden macrophage infiltration. While sepsis is a known complication, abdominal compartment syndrome (ACS) is extremely uncommon. This case underscores the importance of early recognition and timely surgical intervention in managing life-threatening sequelae of XGP. Presentation of Case: A 43-year-old woman with a history of substance abuse and recurrent urinary tract infections presented with septic shock and abdominal distension. Imaging showed classic signs of XGP with mass effect. Initial treatment included antibiotics and nephrostomy drainage. She later underwent a left radical nephrectomy. Histopathology confirmed XGP without calculi, raising the possibility of a methamphetamine-related immune mechanism. The patient recovered fully with preserved renal function. Clinical Discussion: XGP is frequently misdiagnosed due to its nonspecific clinical and radiological features. When complicated by sepsis and intra-abdominal hypertension, the condition can rapidly progress to multiorgan failure. This case highlights the need for high clinical suspicion, multidisciplinary collaboration, and prompt surgical management in severe presentations of XGP. Conclusion: This rare case of XGP complicated by ACS highlights the importance of early diagnosis, timely drainage, and multidisciplinary care. A non-lithogenic origin should be considered in atypical cases, especially in patients with a substance use history.