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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 1  |  Page : 87-90
Atypical Presentation of Xanthogranulomatous Pyelonephritis: A Case Report


Department of Nephrology, Kanoo Kidney Center, Dammam Central Hospital, Dammam, Saudi Arabia

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   Abstract 

Xanthogranulomatous pyelonephritis (XPN) is a rare form of chronic pyelonephritis, which is usually caused by calculous obstructive uropathy. We present a previously healthy 45-year-old housewife, who was admitted to The Dammam Central Hospital, Dammam, Saudi Arabia with left loin pain and increased frequency of micturition of four days duration. She also had icterus and features of disseminated intravascular coagulation. Abdominal ultrasound and computed tomography of the abdomen was suggestive of XPN. She responded well to treatment with antibiotics and nephrectomy. Histology of the resected kidney confirmed a diagnosis of XPN. Our case suggests that the diagnosis of XPN should be kept in mind when a middle-aged female patient presents with unilateral non­functioning hydronephrotic kidney, which is totally distorted, and has enhancing as well as non-enhancing regions on computed tomography.

How to cite this article:
Ghacha R, Youmbissi T J, Sinha AK, Abdelrahman M, Karkar A. Atypical Presentation of Xanthogranulomatous Pyelonephritis: A Case Report. Saudi J Kidney Dis Transpl 2007;18:87-90

How to cite this URL:
Ghacha R, Youmbissi T J, Sinha AK, Abdelrahman M, Karkar A. Atypical Presentation of Xanthogranulomatous Pyelonephritis: A Case Report. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2014 Sep 17];18:87-90. Available from: http://www.sjkdt.org/text.asp?2007/18/1/87/31852

   Introduction Top


Xanthogranulomatous pyelonephritis (XPN) is a rare form of chronic pyelonephritis, which is usually caused by calculous obstructive uropathy. [1] We herein report on a patient with XPN who presented with acute renal failure, hepatic dysfunction and urinary and systemic sepsis without past history of any illness related to the urinary system. The different aspects related to clinical presentation, differential diagnosis and management of XPN are discussed.


   Case Report Top


A previously healthy 45-year-old house- wife was admitted to The Dammam Central Hospital, Dammam, Saudi Arabia with left loin pain and increased frequency of micturition of four days duration. The pain was colicky in nature and was radiating to the lower abdomen. There was no associated vomiting, fever or alteration in the urine volume or color. There was no history of weight loss, anorexia or progressive fatigue. The patient denied any past history of recurrent urinary tract infections, renal stone disease, blood transfusions, diabetes mellitus, hypertension or analgesic abuse. Physical examination showed that she was jaundiced and dehydrated. There was no pallor, leg edema, skin rash of any type or lymphadenopathy. The blood pressure was 120/80 mm Hg lying down and 105/78 mm Hg while sitting, the pulse rate was 100 beats per minute and oral temperature was 36.8°C. Abdominal examination showed a soft and lax abdomen with a ballotable tender mass having ill-defined margins in the left lumbar region. There was no ascites, hepatosplenomegaly or bruit. Physical examination of the head and neck, heart, lungs, and extremities did not show any abnormalities.

Laboratory tests showed hemoglobin of 156 gm/L, hematocrit of 40% with normal cell indices, neutrophilic leukocytosis with shift to the left, thrombocyte count of 9000/cu mm, ESR of. 90 mm/hour, plasma thromboplastin time (PTT) of 48 seconds (control 32 seconds; INR 2) and fibrinogen degradation products (FDP) of 7,500 ng/ml (normal < 250 ng/ml). The serum creatinine was 450 micromol/L, direct bilirubin was 68.4 micromol/L, alanine aminotransferase was 1.6 mmol/L and serum albumin was 25 gm/L. The arterial blood gases (ABG) showed pH of 7.397, PC0 2 of 25 mm Hg, PO 2 of 67 mm Hg, bicarbonate of 16 mmol/L and oxygen saturation of 93.6%. Urinalysis revealed leukocyturia, hematuria without casts and proteinuria.

The differential diagnosis entertained with these results included infection (pyelo­nephritis, sepsis), hepatorenal syndrome and malignancy (renal cell carcinoma). An X-ray of the KUB revealed a radio-opaque shadow corresponding to the ureteric line on the left side. Abdominal ultrasound showed left hydroureteronephrosis, a stone in the left ureter, and thickened cortex in the left kidney. The right kidney and ureter were normal.

A computerized tomography (CT) scan of the abdomen showed a large left kidney (13.5 cm) with totally distorted architecture, multiple enhancing and non-enhancing sectors, thickening of renal capsule, moderate dilatation of the renal pelvis, dilated ureter and no passage of contrast into the ureter. The urine and blood cultures showed  Escherichia More Details coli sensitive to ceftriaxone and aztreonam. Serum antinuclear antibodies, paraprotein and tuberculin skin tests were all negative.

In view of these findings, a diagnosis of XPN of the left kidney with ureteric stone, sepsis, hepatic dysfunction with dissemination intravascular coagulation (DIC) was made; renal cell carcinoma could not be excluded at this stage.

The patient was treated with one session of hemodialysis, and a two-week course of ceftriaxone and aztreonam after which a left­sided nephrectomy was performed. This resulted in normalization of her hepatic and renal functions within seven days. The antibiotics were continued for a total of four weeks. The blood and urine cultures repeated 48 hours after the cessation of antibiotics did not reveal any growth. The resected left kidney was 250 gm in weight, 14 x 5 x 6 cm. in size and showed an adherent capsule and a granular cortex covered with purulent exudates. Cortico­medullary differentiation was lost and the cut surface showed minute abscess and necrosis with markedly dilated pelvis.

Light microscopic examination showed fibrosis and inflammation of the renal parenchyma with vacuolated histiocytes and foam cells surrounding necrotic areas confirming the diagnosis of XPN. There was no evidence of malignancy [Figure - 1],[Figure - 2],[Figure - 3].

Eighteen days after the nephrectomy, the patient complained of dull pain in the left loin without any fever. Ultrasound of the abdomen showed fluid collection in the left kidney space which was drained. The fluid was serosanguinous in character and the culture yielded methicillin resistant Staphy­lococcus aureus (MRSA) for which she received rifampicin and clindamycin.

She was discharged after 35 days of stay in the hospital with normal hepatic and renal functions as well as normal coagulation parameters, ESR and blood cell count. Thus, the final diagnosis was left XPN with left ureteric stone, gram negative sepsis complicated by acute renal failure (ARF), hepatic dysfunction, DIC and MRSA infection of the retroperitoneal space.


   Discussion Top


Xanthogranulomatous pyelonephritis is a rare form of chronic pyelonephritis, which is usually caused by calculous obstructive uropathy, [1] as in our case. The disease is characterized by renal destruction and replacement of renal parenchyma by foam cells (xanthoma cells), i.e. lipid laden macrophages that were first described by Schlagenhaufer in 1916. XPN usually occurs in middle-aged women with a history of recurrent urinary tract infections; the latter was lacking in our patient leading to diagnostic difficulty. The typical presenting symptoms are flank pain, as in this case, and fever. The organism isolated from the urine and blood in our patient was E. coli, a gram­negative organism, although in one-fourth of cases, urine culture may be sterile. The initial finding of left hydroureteronephrosis on renal ultrasound and totally distorted architecture with multiple non-enhancing sectors on the CT scan raised the possibilities of isolated XPN, renal cell carcinoma or their co-existence. [2],[3] After a course of antibiotics, the patient underwent left total nephrectomy, which is the recommended form of treatment for XPN. [4] The rapid recovery that followed nephrectomy showed that the XPN was responsible for most of the uncommon features encountered in this case. XPN has a good prognosis if it is unilateral or in a localized form as is usually seen in children.

In conclusion, our patient had XPN without a prior history of any urinary symptoms and had associated multiple organ involvement that led to diagnostic and treatment challenges. The diagnosis of XPN should be kept in mind when a middle-aged female patient presents with unilateral non-functioning hydro­nephrotic kidney, which is totally distorted, and has enhancing as well as non-enhancing regions on computed tomography.

 
   References Top

1.Parsons MA, Harris SC, Longstaff AJ, Grainger RG. Xanthogranu­lomatous pyelo-nephritis: a pathological clinical and aetio­logical analysis of 87 cases. Diag Histopathol 1983;6: 203-19.  Back to cited text no. 1  [PUBMED]  
2.Goldman SM, Hartman DS, Fishman EK, et al. CT of Xantho­granulomatous pyelo-nephritis: Radiologic- Pathologic correlation: Am J Roentgenol 1984;142:963-9.  Back to cited text no. 2    
3.Huisman TK, Sands JP Jr. Focal xanthogranulomatous pyelo­nephritis associated with renal cell carcinoma. Urology 1992;39:281-4.  Back to cited text no. 3    
4.Perez LM, Thrasher JB, Anderson EE. Successful management of bilateral xanthogranulomatous pyelonephritis by bilateral partial nephrectomy: J Urol 1993; 149:100-2.  Back to cited text no. 4    

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Correspondence Address:
Reda Ghacha
Kanoo Kidney Center, Dammam Central Hospital, P.O. Box 14563, Dammam 31434
Saudi Arabia
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PMID: 17237898

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]



 

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    Abstract
    Introduction
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    References
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