Xanthogranulomatous pyelonephritis: an overview and management guide for clinicians.

Xanthogranulomatous pyelonephritis is a rare and chronic form of pyelonephritis. Patients can present with an array of signs and symptoms including flank pain, fever and weight loss. The insidious nature of its clinical presentation means that it can mimic a neoplastic process. Kidney stone disease, diabetes and female gender are the most common associated factors. Early diagnosis can be difficult, but is vital to avoid sequelae such as abscess formation and involvement of surrounding structures. Management requires a multidisciplinary approach. Definitive resolution can only be achieved through nephrectomy. This article provides an overview and guide to diagnosis, investigation and management.

The use of nephrostomy tubes in optimizing surgical conditions for a patient with xanthogranulomatous pyelonephritis.

Xanthogranulomatous pyelonephritis is a rare variant of pyelonephritis that often requires radical surgery. We report a case of a 51-year-old female patient who was managed with prolonged placement of nephrostomy tubes prior to surgery. This case illustrates the importance of surgical optimization of a poor operative candidate and the use of nephrostomy tubes as a temporizing management strategy.

Ureaplasma urealyticum infection presenting as pyelonephritis and perinephric abscess in an immunocompromised patient.

We report a case of an immunosuppressed 67-year-old woman who presented with fever of unknown origin. Further investigation revealed multiple left renal and perinephric abscesses. These were managed with percutaneous drainage and broad-spectrum antibiotics; however, no clinical improvement resulted. No organism was identifiable on standard microscopy and culture of all drain, blood and urine samples taken. Left nephrectomy with right urinary diversion was performed for sepsis management and to protect the patient's right kidney. Eventually, Ureaplasma species' RNA was detected in the patient's drain fluid using PCR and 16S ribosomal RNA gene sequencing. The patient was treated successfully with targeted antibiotic therapy and underwent extensive rehabilitation following this. Histopathology of the nephrectomy specimen suggested xanthogranulomatous pyelonephritis.

Xanthogranulomatous pyelonephritis in adults: clinical, biological, radiological and therapeutic main findings in diffuse and focal forms. About 42 cases.

INTRODUCTION: Xanthogranulomatous pyelonephritis (XGPN) is a particular form of chronic renal suppuration characterized by the destruction of renal parenchyma and its replacement by a granulomatous tissue containing xanthomatous cells. OBJECTIVE: To report and describe the main clinical, biological, radiological and therapeutic aspects of xanthogranulomatous pyelonephritis in adults in focal and diffuse forms. METHODS: This was a monocentric retrospective study involving 42 cases of XGPN operated and histologically confirmed between 2005 and 2015. We included in our study the patients belonging to any age, of both sexes, with a preoperative CT and whose XGPN diagnosis was confirmed histologically. Clinical, biological, bacteriological, radiological data and surgical procedures were collected and analyzed. RESULTS: The mean age was 50 years (34-79) with a sex ratio of 0.61. The XGPN was diffuse in 88% and focal in 12% of cases. Clinical symptoms were dominated by low back pain (95%), fever (64%) and recurrent urinary tract infections (41%). Urine culture was positive in 67% of cases and Escherichia Coli was the most isolated germ (36%). 64% of patients had inflammatory anemia and 57% of patients had an inflammatory biological syndrome. Ultrasound and CT revealed obstruction of the urinary tract in all cases: a lithiasic cause in 90% of cases and the rest were isolated cases of retroperitoneal fibrosis, abnormal pyelo-ureteral junction, ureteral stenosis and pyelo-ureteric duplicity. A peri-renal abscess and / or psoas was associated in 6 cases (14%). The preoperative diagnosis was often pyonephrosis (50%). Neoplasia was suspected in 4 cases. The treatment consisted in a radical nephrectomy for all patients preceded by drainage of the excretory pathways in 41% of the cases and drainage of a collection in 21% of the cases. CONCLUSION: The XGPN is a rare entity whose diagnosis is difficult. This is suspected on a cluster of clinical and biological arguments and can be evoked on the preoperative CT. The kidney cancer is the main differential diagnosis.

Bear Paw Sign: Xanthogranulomatous Pyelonephritis.

Xanthogranulomatous pyelonephritis is a rare form of chronic pyelonephritis in which the involved areas of the kidneys are destroyed and replaced by foam cells. It usually occurs in immunocompromised middle-aged females with ureteral obstruction or chronic urinary tract infection induced by the formation of renal stones. We herein report the case of a 44-year-old woman, with a history of left kidney staghorn calculi and recurrent urinary tract infections. Abdominal computed tomography showed the 'bear paw' sign, typical appearance of XGP and patient underwent a nephrectomy with uneventful post-operative course.

Xanthogranulomatous pyelonephritis presenting as acute pleuritic chest pain: a case report.

BACKGROUND: 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. CASE PRESENTATION: 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. CONCLUSIONS: 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.

Combination of Surgical Drainage and Renal Artery Embolization: An Alternative Treatment for Xanthogranulomatous Pyelonephritis.

Conventionally, xanthogranulomatous pyelonephritis is treated with antibiotics and drainage of abscess followed by nephrectomy for definitive treatment. Surgical excision of the affected kidney carries risk of significant complications. An alternative treatment modality is described in the form of embolization of the renal artery to devascularise the renal parenchyma and ablate the renal tissue, thus avoiding a major surgical procedure and the significant risks involved.

Xantogranulomatous Pyelonephritis in children.

OBJECTIVE: Xanthogranulomatous Pyelonephritis (XP) is a renal chronic inflammatory disease infrequent during childhood produced by the association of long-term urinary tract obstruction with chronic infection. We report the cases of two children diagnosed of XP. METHODS: One of them consulted for cloudy urine. Hypergammaglobulinemia with the presence of a homogeneous band in the gamma fraction of the proteinogram stood out among laboratory findings. The other case was diagnosed while performing a urologic ultrasound in the follow up of kidney stone disease. In the latter, the renal inflammation was adhered to the perirenal capsule, colonic splenic flexure and descending colon. Both presented mild proteinuria and hematuria. None of them showed clinical sings of affectation of the general status and their renal function laboratory data as well as the infection markers were normal. RESULTS: All the alterations disappeared after performing a nephrectomy of the affected kidney. CONCLUSIONS: We present two cases of XP in a group of age rarely affected by this disease that also showed an uncommon presentation for their age. A high rate of suspicion must be held to diagnose and correctly treat this disease because the grade of renal and extra renal damage is closely related with the extension and duration of the disease.

Xanthogranulomatous pyelonephritis: critical analysis of 18 cases from a rural tertiary care centre of India.

Xanthogranulomatous pyelonephritis is an uncommon disorder of the kidney that is characterised by chronic inflammation and extensive destruction of the involved kidney usually associated with renal calculus and obstruction. It commonly involves the middle aged people with an increased incidence among females and diabetic population. Confirmed diagnosis can be done only after histopathological examination of the diseased nephrectomy specimen. So early suspicion and timely intervention is important to reduce the morbidity. In this retrospective study, we analysed the case records of all patients diagnosed with xanthogranulomatous pyelonephritis in our institute over a period of 11 years. A total 18 patients were treated and diagnosed with xanthogranulomatous pyelonephritis from January 2002 to December 2012. Among them 13 were female and 5 male. Most common clinical presentation was flank pain, fever, dysuria and weight loss. Left kidney was affected in 15 cases and right kidney in 3 cases. All patients were having urinary calculus causing obstruction to the pelvicalyceal system of the kidney. Only 2 patients were diabetic. Urine culture was sterile in all patients. All patients were treated with antibiotics, other supportive measures pre-operatively and ultimate nephrectomy having good recovery. Early suspicion and prompt treatment is necessary in minimising morbidity and mortality from the condition.

Pielonefritis xantogranulomatosa en adultos: presentación de 15 casos clínicos / Xanthogranulomatous pyelonephritis in adults: presentation of 15 clinical cases

Reportar nuestra experiencia en pielonefritis xantogranulomatosa (PXG), para contribuir al conocimiento de la misma. Material y métodos: análisis retrospectivo de las nefrectomías de los pacientes con diagnóstico histopatológico de PXG, entre los años 2002 y 2010. Resultados: se estudiaron 15 casos, 12 mujeres y 3 varones, con una edad promedio de 46,7 años. Todos los pacientes presentaron evolución crónica, malestar general, dolor abdominal y/o lumbar. La fiebre, palidez conjuntival, presencia de litiasis renal y pérdida de peso, fueron síntomas frecuentes. El laboratorio de sangre mostró eritrosedimentación muy incrementada y leucocitosis. La orina se presentó con sedimento alterado mostrando: hematuria, piuria y bacteriuria. En menos de la mitad de los casos el urocultivo presentó desarrollo y en algunas oportunidades con flora polimicrobiana y discrepancia en las recuperaciones por nefrostomías y cultivos convencionales. Los estudios por imágenes mostraron un riñón aumentado de tamaño, con presencia de litiasis en su mayoría y diversas alteraciones estructurales. Conclusiones: la PXG es una enfermedad infrecuente, crónica, benigna infecciosa e inflamatoria que afecta al parénquima renal. Poses predilección por el sexo femenino de mediana edad. El diagnóstico se efectúa ante la sospecha clínica, los estudios de laboratorio, de imágenes y se confirma con la histopatología. El tratamiento definitivo es la nefrectomía...

To report our experience, to contribute to the knowledge about, Xanthogranulomatous Pyelonephritis (XGP). Material and Methods: Retrospective analysis of the nephrectomy of patients with histopathological diagnosis of XGP, between 2002 and 2010. Results: We studied 15 cases, 12 women and 3 men, with an avergage age of 46.7 years. All patientes had chronic evolution, malaise, abdominal pain and/or lumbar spine. Fever, pallor, presence of kidney stones and weight loss were common symptoms. Blood laboratory presented increased erythrocyte sedimentation rate and leukocytosis. The urine was presented showing altered sediment, hematuria, pyuria and bacteriuria. In less than half of the cases the urine culture showed developed, sometimes with polymicrobial flora and discrepancy in recoveries by nephrostomy and conventional cultures. Imaging studies showed an increased kidney size, resence of stones in the most cases and various structural abnormalities. Conclusions: XGP is rare, chronic, infectious and inflammatory beningn disease that affects renal parenchyma and usually presents in middle-aged female. Diagnosis is suspected by clinic, laboratory, imaging studies, and confirmed by histopathology. Nephrectomy is the definitive treatment...

[Refractory urinary tract infections: diagnosis and treatment of xanthogranulomatous pyelonephritis]. / Een hardnekkige urineweginfectie: diagnose en behandeling van xanthogranulomateuze pyelonefritis.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is a rare inflammatory condition of the renal parenchyma that is associated with long-term partial obstruction and infections of the urinary tract. Because its symptoms can be non-specific, a diagnosis is often made with delay. CASE DESCRIPTION: We describe 2 patients with frequently recurring urinary tract infections and urinary tract infections that were difficult to treat. Imaging studies unmasked a diffusely enlarged kidney and renal stones, leading to the diagnosis of xanthogranulomatous pyelonephritis. Both female patients were treated successfully by draining the affected kidney by means of a ureteral double-J catheter along with antibiotic treatment. CONCLUSION: Imaging studies are important in patients with recurrent pyelonephritis or persistent pyelonephritis despite adequate antibiotic treatment; urinary tract obstructions and complications of pyelonephritis such as XGP can then be identified. When XGP is adequately treated by antibiotics and drainage of the affected kidney this can sometimes prevent having to perform a nephrectomy.

Diagnóstico y tratamiento de la pielonefritis xantogranulomatosa / Diagnosis and treatment of xanthogranulomatous pyelonephritis

La pielonefritis xantogranulomatosa es una enfermedad inflamatoria-infecciosa crónica que involucra el riñón. Generalmente se presenta en adultos, más comúnmente en las mujeres jóvenes, y se asocia con litiasis. En la mayoría de los casos es unilateral y se acompaña de destrucción parcial o total del parénquima renal. Los hallazgos más frecuentes del examen físico son: fiebre, dolor lumbar o abdominal o ambos, masa palpable, pérdida de peso y antecedentes de infecciones urinarias recurrentes. La tomografía computarizada es el método más comúnmente empleado para su diagnóstico. Inicialmente se la puede confundir con un tumor. En los análisis de laboratorio se observa anemia, eritrosedimentación muy acelerada, leucocitosis y piuria en el sedimento urinario. El diagnóstico de certeza es el histopatológico. El tratamiento antibiótico evita las complicaciones sépticas, pero la terapia de elección es la nefrectomía total o parcial. Nuestro objetivo es actualizar esta enfermedad infrecuente, tanto en sus formas de presentación, fisiopatogenia, manifestaciones clínicas, como su diagnóstico y tratamiento.

Stage III xanthogranulomatous pyelonephritis treated with antibiotherapy and percutaneous drainage.

Xanthogranulomatous pyelonephritis (XPN) is a rare inflammatory condition usually secondary to chronic obstruction caused by nephrolithiasis and resulting in infection and irreversible destruction of the renal parenchyma. Its standard therapy consists of total or partial nephrectomy. A case of stage III xanthogranulomatous pyelonephritis treated with antibiotherapy and percutaneous drainage is presented in this paper.

Xanthogranulomatous pyelonephritis.

Xanthogranulomatous pyelonephritis is an uncommon chronic destructive granulomatous process of renal parenchyma in association with long-term urinary tract obstruction and infection. It affects females more often than males, with a wide range of age, from newborn to elderly. Almost all patients are symptomatic and the most common symptoms are flank or abdominal pain, lower urinary tract symptoms, fever, palpable mass, gross hematuria, and weight loss. The common laboratory findings are leukocytosis and anemia. Urine cultures most often reveal Escherichia coli and Proteus mirabilis . Computed tomography is the mainstay of diagnostic imaging for xanthogranulomatous pyelonephritis. Imaging studies may demonstrate diffuse or focal form. Histologically, xanthogranulomatous pyelonephritis presents a granulomatous inflammatory infiltrate composed of neutrophils, lymphocytes, plasma cells, xanthomatous histiocytes, and multinucleated giant cells. The differential diagnosis includes clear cell renal cell carcinoma, papillary renal cell carcinoma, sarcomatoid renal cell carcinoma, leiomyosarcoma, malakoplakia, and megalocytic interstitial nephritis. Both antibiotics and surgery can be treatment options depending on the patient's disease status.

Xanthogranulomatous pyelonephritis in childhood: diagnosis difficulties and success of conservative treatment.

BACKGROUND: Xanthogranulomatous pyelonephritis is an uncommon form of chronic pyelonephritis rarely seen in children. Preoperative diagnosis is difficult and management is still obscure. AIM: The authors report on a focal pseudotumoral case and discuss therapeutic modalities. CASE: A 2-year-old boy was admitted for a 2-weeks history of fever and right flank pain. Physical examination revealed a right lumbar mass. White blood cell count was 12.800/mm3 and C reactive protein elevated (50 mg/l). The urine culture was negative. Ultrasonography and computerized tomography demonstrated a focal multilocular cystic mass of the lower polar of right kidney, measuring 80 millimeters, with peripheral enhancement and perirenal involvement. At surgical exploration, a huge abscess of the lower polar of the kidney with dense adhesions to adjacent structures were noted. Excision of the collection and local drainage were performed. Pathologic examination of biopsy specimen confirmed the diagnosis of xanthogranulomatous pyelonephritis. Intraoperative culture was positive for Escherichia coli. Successful treatment of the lesion was achieved with adjunctive antibiotic therapy. The postoperative course was uneventful over a follow-up period of 2 years. CONCLUSION: Xanthogranulomatous pyelonephritis should be considered in the differential diagnosis of a renal mass. A first-line conservative treatment must be strongly recommended in pediatric focal cases.

Xanthogranulomatous pyelonephritis in a horse-shoe kidney.

Xanthogranulomatous pyelonephritis (XGPN) represents an unusual suppurative granulomatous reaction to chronic infection, often in the presence of chronic obstruction from a calculus. We present a case of XGPN in a horse shoe kidney in an adult. Hemi-nephrectomy of the involved side was followed by clinical improvement. The case highlights the importance of early hemi-nephrectomy in XPGN with horse shoe kidney.

Xanthogranulomatous pyelonephritis in pediatric patients: case report and review of literature.

Xanthogranulomatous pyelonephritis (XPN) is an unusual and rare form of chronic renal suppuration. XPN is often mistaken for renal malignancy; hence nephrectomy is commonly performed and the diagnosis confirmed by histopathology. Recent advances in imaging have allowed the radiological features to be characterized, such that routine nephrectomy is avoided. Approximately 240 cases of XPN have been reported in children. We report a 17-year-old female who presented with a 2-month history of increasing abdominal pain and intermittent episodes of increased frequency and dysuria. Plain antero-posterior radiograph of the abdomen revealed a left staghorn calculus. Computed tomography scan with intravenous contrast revealed a low-density inflammatory area and reduced cortical dye uptake on the left renal parenchyma as compared to the opposite side. A dimercapto-succinic acid renal scan revealed that the affected kidney contributed 18% of differential function. A diagnosis of XPN was made. The patient underwent percutaneous nephrostomy tube placement in an attempt to salvage the kidney. The patient eventually failed conservative management. Our review of the literature suggests that medical management has worked in some focal XPN cases, but has not been sufficiently tested in diffuse XPN.