Emphysematous Pyelonephritis: A Case Report
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Introduction: Emphysematous pyelonephritis is a particularly severe form of the disease that is associated with the production of gas in renal and perinephric tissues and occurs almost exclusively in diabetic patients. It is rapidly progressive, requiring appropriate therapy to salvage the infected kidney.
Case Report: A 48-year-old female who had been diagnosed as type 2 diabetes mellitus 4 years back, on irregular treatment, came to us in a delirious state with epigastric pain and vomiting. She was diagnosed with diabetic ketoacidosis and was managed in the intensive care unit (ICU). After urine tests and imaging studies such as ultrasound and computed tomography (CT) of abdomen and pelvis were done, a diagnosis of emphysematous pyelonephritis was made. Patient was treated aggressively with antibiotics. Patient improved during the stay without requiring any surgical intervention.
Conclusion: This case provides an insight into a rather uncommon disease entity occurring most commonly in diabetic patients called emphysematous pyelonephritis. It is important for us to consider this as one of the diagnosis in cases of urinary tract infection and diabetes. Early goal directed medical therapy for sepsis is a valuable alternative to circumvent an upfront emergent nephrectomy, except in cases where a fulminant infection may be present at the time of admission or develop later despite a conservative line of therapy.
Emphysematous pyelonephritis (EPN) is a particularly severe form of the disease that is associated with the production of gas in the renal and perinephric tissues and occurs almost exclusively in diabetic patients. Other conditions include immune-incompetence and urinary tract obstruction. It is rapidly progressive, requiring appropriate therapy to salvage the infected kidney. EPN presents a medical challenge in terms of diagnosis, pathogenesis, classification, and management. Therapeutic measures should be applied immediately after diagnosis. The prognosis also varies according to the authors and situations, with a mortality of less than 20% to more than 80%.[3,4] It is caused by gas-forming organisms, most commonly Escherichia coli (E. coli), in addition to Klebsiella, Clostridium, Candida, Aspergillus, Cryptococcus, and Amoeba.
Immediate resuscitation, use of broad-spectrum antibiotics, percutaneous and surgical drainage, and emergent or delayed nephrectomy are therapeutic options that should be applied in a timely fashion. EPN is a life-threatening condition that carries a bad prognosis. Early diagnosis is essential for a positive outcome. Therapy and management of emphysematous pyelonephritis has not been discussed much in standard medical textbooks.
A 48-year-old female who is a known diabetic for the last 4 years, on irregular treatment for the same, came to the Casualty in a delirious state with complaints of vomiting and upper abdominal pain for the past 2 days. On examination, she was febrile (102 degrees Fahrenheit), tachycardic, and with epigastric tenderness. Abdominal examination revealed bowel sounds, no organomegaly or evidence of free fluid. Fundus examination showed non-proliferative diabetic retinopathy. Random blood sugar was 480 mg/dL and urine was positive for acetone. Arterial blood gas showed metabolic acidosis. She was subsequently admitted in the Intensive Care Unit (ICU). Treatment for diabetic ketoacidosis was started along with empirical antibiotic cover. Subsequent investigations showed markedleukocytos is and elevated urea levels. Ultrasound abdomen and pelvis showed dirty shadows in pelvicalyceal region, thus CT abdomen and pelvis (plain) was done which confirmed the diagnosis of emphysematous pyelonephritis (Figures 1, 2, and 3).
Culture and sensitivity reports showed Klebsiella and the patient was started on piperacillin/ tazobactam. Patient slowly recovered and was discharged in a hemodynamically stable condition with insulin therapy for diabetes.
EPN is a severe necrotizing infection characterized by bacterial production of gas within the renal parenchyma. The conditions required for the generation of EPN are:
1) the presence of pathogenic bacteria capable of mixed acid fermentation
2) high levels of glucose in tissue
3) impaired tissue perfusion
These factors can work collectively resulting in rapid progression of the disease; therefore, the level of suspicion should increase in conjunction with number of predisposing conditions. For example, local tissue ischemia in the presence of gas-forming bacteria will exacerbate tissue destruction, encourage purulent infection, and inhibit the removal of locally produced gas.
Diabetes is currently the leading cause of end stage renal disease. The patient’s compromised immune system rendered her unable to fight off infection from these gas-forming bacteria that result in EPN in this case. The pathogenesis of gas formation requires pathogenic bacteria capable of mixed acid fermentation, a hyperglycemic environment, and localized tissue ischemia. Because a hyperglycemic environment is one of the requirements in gas formation, it only makes sense that diabetes is a significant predisposing factor.
It has been estimated that up to 95% of EPN cases have an underlying uncontrolled diabetes mellitus. Further more, hyperglycemia in association with impaired blood supply to the kidneys from vasculopathy, both of which are prevalent in diabetic patients, facilitates the process of anaerobic metabolism.
Current evidence suggests that females are more susceptible to EPN because they are also more susceptible to urinary tract infections.[9-11] E. coli is noted to be a very common pathogen in EPN.[9-14] Klebsiella however is not so common.
The clinical approach to treating patients with EPN has changed over the years. Currently, due to advances in medical imaging, interventional radiology, newer more effective antibiotic therapy, and readily available intensive care integrated with dialytic support, patients with EPN have much better outcomes. Managing EPN more conservatively has thus become the standard of care. In patients with extensive/fulminant disease with hemodynamic compromise, it has been determined that together with fluid resuscitation and antibiotics, immediate nephrectomy should not be delayed for the successful management of EPN.[16-18]
EPN is a severe acute necrotizing renal parenchymal infection caused by gas-forming organisms, among others. Emphysematous pyelonephritis is a particularly severe form of the disease that is associated with the production of gas in renal and perinephric tissues and occurs almost exclusively in diabetic patients. It possesses great risk and possesses higher mortality than conventional cases of pyelonephritis. It is rapidly progressive, requiring appropriate therapy to salvage the infected kidney.
EPN has no specific signs and symptoms, and it can be present even in the absence of sepsis. EPN should be suspected in patients who are not responding to antibiotics, in which there is unexplained abnormal gas formation in the body, especially in the immuno-compromised, like the diabetic with poor glycemic control and transplant recipients on immuno-suppression. Hence, it is important to consider the diagnosis of emphysematous pyelonephritis in diabetic patients with urinary infections and treat it with immediate and aggressive therapy. Moreover, the need for physicians to know how to manage a case of emphysematous pyelonephritis is also important. Though not a common disease, it can be deadly.
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