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Published on: August 15, 2019
Metastatic xanthogranulomatous pyelonephritis managed with immunosuppression
Julian Daniell1, Eugenia Pedagogos2, Anastasia Chrysostomou3
1Royal Melbourne Hospital, Parkville, Victoria, Australia.
Xanthogranulomatous disease, a rare condition, can recur after initial treatment. Immunosuppressive therapy, including glucocorticoids and azathioprine, effectively managed a patient
Area of Science:
- Nephrology
- Oncology
- Immunology
Background:
- Xanthogranulomatous pyelonephritis (XGP) is a rare, chronic granulomatous inflammation of the kidney.
- This case presents a rare extra-renal manifestation of xanthogranulomatous disease.
- The patient had a history of type 2 diabetes mellitus, hypertension, and active smoking.
Purpose of the Study:
- To report a rare case of recurrent xanthogranulomatous disease with extra-renal involvement.
- To discuss the management of recurrent xanthogranulomatous disease with immunosuppressive therapy.
Main Methods:
- Diagnosis of xanthogranulomatous pyelonephritis via nephrectomy.
- CT imaging for detection of pulmonary and pancreatic lesions.
- Histological confirmation of xanthogranulomatous disease.
- Treatment with glucocorticoids and azathioprine.
Main Results:
- Initial diagnosis of xanthogranulomatous pyelonephritis after nephrectomy for a renal mass.
- Recurrence of xanthogranulomatous disease with pulmonary and pancreatic lesions 3 years post-diagnosis.
- Successful management with glucocorticoids and azathioprine, leading to stable renal function and no active inflammation for 2 years.
Conclusions:
- Xanthogranulomatous disease can manifest with extra-renal involvement and recur.
- Glucocorticoids and azathioprine are effective in managing recurrent xanthogranulomatous disease.
- Long-term immunosuppressive therapy can lead to disease stability.
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