| |
|
|
| Year : 2010 | Volume
: 21
| Issue : 4 | Page : 720-723 |
|
| Amyloidosis secondary to xanthogranulomatous pyelonephritis: A rare association |
|
|
Rajpal Singh Punia1, Neerja Dhingra1, Harsh Mohan1, Sanjay D'Cruz2
1 Department of Pathology, Government Medical College and Hospital, Chandigarh, India
2 Department of Internal Medicine, Government Medical College and Hospital, Chandigarh, India
Click here for correspondence address and email
| Date of Web Publication | 26-Jun-2010 |
|
|
 |
|
 Abstract | | |
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 | |  |
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 | | |
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 | | |
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 | | |
| 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.  |
| 2. | Val-Bernal JF, Garijo MF. Xanthogranulomatous pyelonephritis associated with systemic amyloidosis-case report. Int J Surg Pathol 1998;6 (1):29-35. |
| 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. |
| 4. | Kim JC. US and CT findings of xanthogranulomatous pyelonephritis. Clin Imaging 2001; 25 (2):118-21. |
| 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. |
| 6. | Garber BB, Cendron M, Cohen R, Whitmore KE. Xanthogranulomatous pyelonephritis and amyloidosis: a rare association. J Urol 1989; 142(1):114-6. |
| 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. |
| 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. |
| 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. |
| 10. | Almirall J, Lopez T, Saez A, Gratacos J, Prats J. Systemic amyloidosis secondary to xanthogranulomatous pyelonephritis. Nefrologia 2001; 21(5):505-8. |
| 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. |
Correspondence Address:
Neerja Dhingra
Department of Pathology, Government Medical College and Hospital, Sector 32-A, Chandigarh 160030
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 20587879 
[Figure 1], [Figure 2] |
|
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 2889 | | | Printed | 111 | | | Emailed | 0 | | | PDF Downloaded | 418 | | | Comments | [Add] | | |
|
|
