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The n e w e ng l a n d j o u r na l of m e dic i n e

Images in Clinical Medicine

Chana A. Sacks, M.D., Editor

Xanthogranulomatous Pyelonephritis

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Lucas X. Marinacci, M.D. 73-year-old woman presented to the emergency department
Ivy Rosales, M.D. with a 10-day history of fever and lethargy. Her medical history included
Massachusetts General Hospital type 2 diabetes, recurrent urinary tract infection, and weight loss of ap-
Boston, MA proximately 13.5 kg during the previous year. Laboratory studies revealed a white- cell count of 14,290 per cubic millimeter (reference range, 4500 to 11,000) and a
serum creatinine level of 1.3 mg per deciliter (115 µmol per liter; reference range,
0.6 to 1.5 mg per deciliter [53 to 133 µmol per liter]); examination of the urine
sediment revealed 50 to 100 white cells per high-powered field, but a culture was
negative for bacteria. Computed tomography of the abdomen performed with the
intravenous administration of contrast material (Panel A) revealed an enlarged
right kidney with staghorn calculus in the ureteropelvic junction, cortical atrophy,
and calyceal dilatation — an appearance referred to as a “bear paw sign.” Intrave-
nous antibiotic agents were initiated, and percutaneous nephrostomy was per-
formed. Culture of the drainage grew Proteus mirabilis. After a radionucleotide scan
showed that renal function was absent on the right side, nephrectomy was per-
formed. Gross pathological analysis showed a staghorn calculus (Panel B, arrow),
dilated calyces, cystic cavities, and yellow tissue. Histologic testing revealed fibro-
sis and chronic granulomatous inflammatory infiltrate with lipid-laden foamy
macrophages and necrotic debris. Xanthogranulomatous pyelonephritis was diag-
nosed. Xanthogranulomatous pyelonephritis, a form of chronic pyelonephritis, is
typically associated with obstructing calculi and recurrent urinary tract infection.
Complications may include abscesses and fistulae. Loss of renal function is common,
and nephrectomy is the definitive treatment. The patient was discharged 5 days
after surgery and was feeling well with stable renal function at 1 month of follow-up.
DOI: 10.1056/NEJMicm1710400
Copyright © 2018 Massachusetts Medical Society.

940 n engl j med 378;10 March 8, 2018

The New England Journal of Medicine

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