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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 4  |  Page : 720-723
Amyloidosis secondary to xanthogranulomatous pyelonephritis: A rare association

1 Department of Pathology, Government Medical College and Hospital, Chandigarh, India
2 Department of Internal Medicine, Government Medical College and Hospital, Chandigarh, India

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Date of Web Publication26-Jun-2010


Xanthogranulomatous pyelonephritis and secondary systemic amyloidosis are re­latively common pathologies with innumerable cases being reported now and then. However the association of these entities is an extremely uncommon occurrence with only ten cases described in the literature. Clinical remission of amyloidosis develops in a majority of these patients after re­moval of the renal lesion. We present a case of this rare association in a young female who under­went nephrectomy for a non-functioning kidney. A histopathological diagnosis of xanthogranu­lomatous pyelonephritis was made. In addition there were deposits of amyloid in the glomeruli and the interstitial blood vessels.

How to cite this article:
Punia RS, Dhingra N, Mohan H, D'Cruz S. Amyloidosis secondary to xanthogranulomatous pyelonephritis: A rare association. Saudi J Kidney Dis Transpl 2010;21:720-3

How to cite this URL:
Punia RS, Dhingra N, Mohan H, D'Cruz S. Amyloidosis secondary to xanthogranulomatous pyelonephritis: A rare association. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2022 Aug 16];21:720-3. Available from: https://www.sjkdt.org/text.asp?2010/21/4/720/64659

   Introduction Top

Xanthogranulomatous pyelonephritis (XGP) is an atypical form of chronic pyelonephritis characterized by the formation of inflamma­tory renal masses rich in macrophages loaded with lipids. [1] The exact etiology of XGP is un­known, but it is generally accepted that the disease process requires long-term renal obs­truction and infection, which produces slow destruction of the renal parenchyma. Staghorn calculi figure prominently as a cause of obs­truction. The condition has a common association with Proteus or  Escherichia More Details coli infection. Pseudomonas species have also been impli­cated. [1],[2] Reactive systemic amyloidosis is well known to be associated with a number of chro­nic inflammatory diseases. [3] The association of xanthogranulomatous pyelonephritis and sys­temic amyloidosis is extremely rare. To the best of our knowledge, till date there have been only ten reports of this association. Here­in, we describe a case of this uncommon asso­ciation for the purpose of obtaining its wider recognition.

   Case Report Top

A 25-year-old non diabetic woman presented with pain in the right flank and recurrent urinary tract infection of six months duration. She had a history of renal colic three months back, which was managed conservatively at a private nursing home. Physical examination revealed pallor, tenderness in the right flank, bilateral pitting edema of lower limbs and a blood pressure of 110/70 mmHg. Hemogram revealed hemoglobin of 6.7 g/dL, total leuco­cyte count of 13,600/μL (neutrophils 78%; lymphocytes 18%; monocytes, 02%; and eosi­nophils, 02%), platelet count of 2.9 Χ 10 5/ μL and ESR of 18 mm first hour by Westergren method. The peripheral blood smear showed normochromic normocytic red blood cells and did not reveal any atypical cells. Biochemical analysis revealed fasting blood glucose of 86 mg/dL, normal serum electrolytes, calcium, phosphate and alkaline phosphatase, an elevated BUN of 29 mg/dL, serum creatinine of 2.8 mg/dL, total serum protein of 7.0g/L, serum albumin of 2.5 g/L, globulin of 4.5g/L and serum cholesterol of 265 mg/dL. Urine ana­lysis showed albumin ++, microscopy revealed field full of pus cells. Twenty four hour uri­nary protein excretion was 4.5 g. Urine did not show presence of Bence-Jones proteins. Serum and urine electrophoresis did not show any M­band. Skeletal survey including X-ray chest, pelvis, skull and spine did not reveal anylytic lesions. Urine culture grew Escherichia coli with colony count greater than 10 5 /mm 3 on two occasions. Blood culture was sterile. Ultrasound abdomen revealed an enlarged hydronephrotic right kidney containing a staghorn calculus in the pelvis. The right kidney was non-functio­nal on excretory urography. The GFR on the right side was 3 mL/min and on the left was 38 mL/min on Tc DTPA scan. The patient under­went right nephrectomy. The post-operative period was uneventful and she was discharged from the hospital 15 days after surgery.

Grossly the resected specimen measured 16 Χ 12 Χ 6.5 cm. The capsule was adherent and could not be stripped off. On cut section the pelvi-calyceal system was markedly dilated and the kidney was converted into a large sac with a staghorn calculus at the pelvis. Yello­wish nodules were apparent on the cut surface. Histopathological examination revealed exten­sive xanthogranulomatous inflammation of the kidney with abundant lipid-laden foamy mac­rophages admixed with acute and chronic in­flammatory cells and multinucleated giant cells [Figure 1]. No Michaelis-Gutmann bodies were seen. The residual renal parenchyma showed deposition of eosinophilic acellular hyaline material in the glomeruli [Figure 2] and in the walls of the blood vessels. The nature of eosi­nophilic material was confirmed as amyloid on Congo red staining which had characteristic apple green birefringence under polarized light. The permanganate digestion technique suggested the diagnosis of an AA-type amyloid. At the time of discharge the urinary examination re­vealed mild proteinuria. Thereafter the patient did not report to the hospital and was lost to follow up.

   Discussion Top

Xanthogranulomatous pyelonephritis (XGP) is an unusual and relatively rare form of chro­nic pyelonephritis. It is usually the result of chronic obstruction to the urinary outflow and infection. Staghorn calculi are the most com­mon cause of obstruction that may be seen in 75 to 86% of these patients, but are not essen­tial to make the diagnosis. [4] The disease is associated with infection by Proteus mirabilis, Escherichia coli, Klebsiella, Enterobacter and Pseudomonas species. [1],[2] XGP is more common in women than men and is usually noted in the fifth and sixth decades of life. Treatment con­sists of total or partial nephrectomy. [5]

The association of XGP and systemic amy­loidosis is an extremely rare occurrence. In an extensive Medline search, we could find only ten cases of this association. [2],[5],[6],[7],[8],[9],[10],[11] Bilbao Garay et al [11] estimated that less than 1% of all the XGP cases are complicated by amyloidosis. The combination of these entities has been reported in childhood as well as in adults. Reactive systemic amyloidosis is well known to be associated with a number of chronic in­ flammatory diseases. [2],[3] The diseases that are commonly implicated as the underlying causes of reactive systemic (secondary) amyloidosis include tuberculosis, leprosy, infected decu­bitus ulcers, chronic osteomyelitis, syphilis, malaria and bronchiectasis. Systemic amyloi­dosis is also known to complicate rheumatoid arthritis, ankylosing spondylitis and inflamma­tory bowel disease. It may also occur in asso­ciation with non-immunocyte derived tumors, the most common being renal cell carcinoma and Hodgkin's disease. [3] These chronic infla­mmatory disease states result in elevated levels of serum amyloid associated protein (SAA) which ultimately gets deposited in tissues in the form of AA amyloid. Our patient did not have any chronic inflammatory conditions or neoplastic processes known to be associated with amyloidosis. Amyloidosis was in all probability secondary to XGP in the index case.

These patients of XGP having coincident systemic amyloidosis most commonly present with nephrotic syndrome or chronic renal fai­lure. Clinical remission of amyloidosis deve­lops in a majority of the patients after removal of the renal lesion. [3] Nephrotic syndrome was the initial presentation in the present case; in addition she had moderate renal insufficiency. At the time of discharge from the hospital, the patient had mild proteinuria. We cannot com­ment on the further course of the proteinuria as our patient was lost to subsequent follow up.

In conclusion, we have presented this case to obtain a wider recognition of XGP as an un­derlying cause of secondary amyloidosis. Though XGP is a common condition, its com­bination with amyloidosis is extremely rare. The awareness of this uncommon association is important so that amyloid deposits are looked for in cases of XGP or in other chronic inflammatory destructive lesions of the kidney.

   References Top

1.Parsons MA, Harris SC, Longstaff AJ, Grainger RG. Xanthogranulomatous pyelonephritis: A pathological, clinical and aetiological analysis of 87 cases. Diagn Histopathol 1983;6(3-4): 203-19.  Back to cited text no. 1      
2.Val-Bernal JF, Garijo MF. Xanthogranulomatous pyelonephritis associated with systemic amy­loidosis-case report. Int J Surg Pathol 1998;6 (1):29-35.  Back to cited text no. 2      
3.Sipe JD, Cohen AS. Amyloidosis. In: Kasper et al eds. Harrison's principles of internal me­dicine, 16 th edition. McGraw Hill, 2005:2024-­9.  Back to cited text no. 3      
4.Kim JC. US and CT findings of xanthogra­nulomatous pyelonephritis. Clin Imaging 2001; 25 (2):118-21.  Back to cited text no. 4      
5.Lauzurica R, Felip A, Serra A, et al. Xantho­granulomatous pyelonephritis and systemic amyloidosis: report of 2 new cases and the natural history of this association. J Urol 1991; 146(6):1603-6.  Back to cited text no. 5      
6.Garber BB, Cendron M, Cohen R, Whitmore KE. Xanthogranulomatous pyelonephritis and amyloidosis: a rare association. J Urol 1989; 142(1):114-6.  Back to cited text no. 6      
7.Querfeld U, Waldherr R, Twittenhoff W, Mohring K, Scharer K. Generalized amyloidosis secondary to xanthogranulomatous pye­lonephritis. Eur J Pediatr 1986;145(6):565-8.  Back to cited text no. 7      
8.Noyan A, Duman N, Gonlusen G, Anarat A, Tuncer I. Amyloidosis secondary to xantho­granulomatous pyelonephritis: an unusual case. Pediatr Nephrol 1995;9(2):251.  Back to cited text no. 8      
9.Islek I, Baris, Albayrak D, Buyukalpelli R, Sancak R. Chronic nephrotic syndrome and chronic renal failure by amyloidosis secondary to xanthogranulomatous pyelonephritis. Clin Nephrol 1998;49(1):62-5.  Back to cited text no. 9      
10.Almirall J, Lopez T, Saez A, Gratacos J, Prats J. Systemic amyloidosis secondary to xantho­granulomatous pyelonephritis. Nefrologia 2001; 21(5):505-8.  Back to cited text no. 10      
11.Bilbao Garay J, Zapatero Gaviria A, Dominguez Frajo P, Llorente Abarca C, Fernandez Juarez G. Amyloidosis secondary to xanthogranulomatous pyelonephritis: a case report and review of the literature. Rev Clin Esp 2007;207(1):55-6.  Back to cited text no. 11      

Correspondence Address:
Neerja Dhingra
Department of Pathology, Government Medical College and Hospital, Sector 32-A, Chandigarh 160030
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Source of Support: None, Conflict of Interest: None

PMID: 20587879

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