Rare form of chronic pyelonephritis and represents a chronic granulomatous disease resulting in a non-functioning kidney

Aetiology

• Chronic granulomatous process believed to be the result of subacute/chronic infection inciting a chronic but incomplete immune reaction

Pathophysiology

• Inflammatory but has all radiological features of malignancy

Clinical features

• Typically vague, consisting of constitutional symptoms such as malaise, weight loss and low-grade fever
• Haematuria and flank pain are sometimes encountered

Investigations

• Imaging: x-ray, USS, CT (most useful), MRI

Management

• Surgical nephrectomy is usually curative