Xanthogranulomatous pyelonephritis presenting as acute pleuritic chest pain: a case report
© The Author(s). 2017
Received: 14 January 2017
Accepted: 23 March 2017
Published: 12 April 2017
Xanthogranulomatous pyelonephritis is a rare and serious manifestation of chronic kidney inflammation that can be life-threatening if not recognized and treated appropriately, often with antibiotics and surgery. Affected patients are most commonly females in their fifth or sixth decade of life with a background of obstructive uropathy, nephrolithiasis, or recurrent urinary tract infections who present with vague nonspecific symptoms.
A 43-year-old woman of Russian ethnicity with a history of nephrolithiasis presented to our emergency department with new left-sided pleuritic chest pain amid a 6-week history of constitutional symptoms including fevers, night sweats, and 7 kg of weight loss. Workup for acute coronary syndrome and pulmonary embolism in our emergency department was negative. Given that she was clinically unwell, she was admitted to internal medicine to expedite workup for the cause of her symptoms. A broad differential diagnosis for various infectious, inflammatory/autoimmune, and neoplastic processes was considered. Based on classic radiographic and histopathologic findings, she was ultimately diagnosed with xanthogranulomatous pyelonephritis of her left kidney, which was a direct consequence of chronic inflammation. This inflammation exhibited spread to local tissues and across her left hemidiaphragm, resulting in a unilateral pleural effusion which explained her chest discomfort. She was treated with antibiotics administered intravenously and urgent total nephrectomy with a good functional outcome.
Our case illustrates an uncommon but clinically important do-not-miss diagnosis that underlies a common clinical presentation of pleuritic chest pain. The case underscores the importance of maintaining a broad differential diagnosis and organized approach when treating patients with undifferentiated clinical presentations.
KeywordsXanthogranulomatous pyelonephritis Pyelonephritis Pleural effusion Pleuritic chest pain Pleurisy
Xanthogranulomatous pyelonephritis (XGP) is a rare manifestation of chronic kidney inflammation that can be life-threatening if not recognized early and treated appropriately. The prototypical patient affected by this condition is a female in her fifth or sixth decade of life with a background of obstructive uropathy presenting with nonspecific constitutional symptoms . Diagnosis of XGP is usually made radiographically and later confirmed by histopathology. Treatment most often involves a combination of surgery and antibiotics [1, 2]. Here, we describe a very atypical case of XGP in which the patient’s presenting symptom was acute-onset pleuritic chest pain.
Hb 88 g/L
MCV 70 fL
Na 133 mmol/L
K 3.9 mmol/L
Cl 98 mmol/L
HCO3 − 22 mmol/L
Urea 2.9 mmol/L
Calcium 2.12 mmol/L
Phosphate 1.10 mmol/L
Magnesium 0.88 mmol/L
TSH 3.41 mIU/L
Liver function panel
LD 141 U/L
ALT 22 U/L
ALP 207 U/L
GGT 110 U/L
Total bilirubin 7 umol/L
Lipase 16 U/L
Erythrocyte sedimentation rate
Troponin T (high-sensitivity)
She was empirically started on intravenously administered broad-spectrum antibiotics with piperacillin-tazobactam 3.375 g intravenously administered every 6 hours and urgent urological consultation was obtained. She ultimately underwent a successful laparoscopic total left nephrectomy. No definitive microbiologic diagnosis was ever made on cultures of urine, blood, and surgical specimens, even after incubation for the maximum time period of 24 hours, 5 days, and 4 weeks, respectively. Empiric piperacillin-tazobactam was continued for 7 days preoperatively and 7 days postoperatively and a decision was made to discharge the patient home on an additional 7-day course of orally administered cefixime 200 mg daily. She has been medically and functionally well for nearly 8 months after hospitalization.
XGP is a chronic and severe retroperitoneal infectious/inflammatory process that manifests as chronic destructive granulomatous inflammation of renal parenchyma often resulting in complete loss of function in the affected kidney. This inflammation may invade and spread to adjacent structures, most commonly the gastrointestinal tract, urinary tract, and skin . As previously mentioned, patients are typically females in their fifth or sixth decade of life; patients often present with flank pain, lower urinary tract symptoms, as well as constitutional symptoms such as recurrent fevers and weight loss . Although the pathogenesis of XGP is not known, almost all cases occur in the setting of obstructive uropathy, nephrolithiasis, and/or recurrent urinary tract infections . Diagnosis of XGP requires a combination of typical clinical symptoms and classic imaging features such as the loss of normal kidney contour and dilated renal calyces surrounding a paradoxically contracted renal pelvis (“bear’s paw sign” ) in patients with a history of urinary tract disease. Definitive diagnosis requires histopathological examination of specimens. Only recently has the coexistence of malakoplakia been reported in rare cases, suggesting that XGP and malakoplakia may in fact be two entities on the same spectrum of disease [5, 6].
Once a diagnosis of XGP is established, treatment involves a combination of systemic broad-spectrum antibiotics as well as surgical nephrectomy (partial versus total depending on extent of kidney involvement) for source control [7, 8]. Given the rarity of XGP, there are no guidelines available to inform optimal antimicrobial selection, duration, and timing of surgery; these decisions are often made at the discretion of the attending physician. Empiric broad-spectrum antibiotics (which should cover for Staphylococcus aureus and Pseudomonas aeruginosa) are continued until culture data are available to guide an appropriate stepdown regimen. If adequate source control is achieved, antibiotics are typically continued for 1 to 2 weeks postoperatively [9, 10]. As shown in two relatively large case series of 41 and 26 patients, offending pathogens are usually found by culturing resected tissues, with the two most common culprit organisms being Escherichia coli and Proteus mirabilis [10, 11]. In exceedingly rare instances of focal disease (defined as less than 10% kidney involvement ), XGP has been successfully treated non-surgically with prolonged courses of antibiotics and followed with serial ultrasonography. This was demonstrated in a case of a 73-year-old woman with leukemia who was treated for XGP with 2 months of intravenously administered antibiotics due to being at high surgical risk . Other than in patients who are poor surgical candidates, this approach may also have applicability to renal transplant recipients with focal disease for the purposes of allograft salvage.
To the best of our knowledge, this is the first reported case of a patient with XGP presenting with pleuritic chest pain as a chief symptom. Her pain was explained by local spread to and inflammatory involvement of her left hemidiaphragm, in fact an atypical presentation of this disease. Nonetheless, this case reaffirms the need to consider a broad differential diagnosis in the medically undifferentiated patient; in patients with a history of lower urinary tract pathology presenting with abdominal, flank, or even chest pain amid constitutional symptoms, it may be reasonable to consider retroperitoneal infectious/inflammatory processes such as XGP in the differential diagnosis and, in the correct clinical context, adopt a low threshold to order imaging studies of the abdomen/pelvis and obtain early urological consultation.
White blood cell
Availability of data and materials
JC, RK, KL, and MS all contributed to the writing and iterative editing of this case report. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
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Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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