Xanthogranulomatous pyelonephritis, and emphysematous pyelonephritis: Two rare conditions in one patient

Abstract

Introduction

What makes this case unique are combined features of two rare renal disease processes in one patient within one kidney which are Xanthogranulomatous pyelonephritis,and emphysematous pyelonephritis.

Case presentation

The patient had main general symptoms like fever, chills, fatigue,and right abdominal pain. He had urea = 77, White Blood Cells = 15,46, and urine culture was positive for Klebsiella. He had right total nephrectomy as a therapeutic intervention.

Conclusion

It is important to note that it could we have two diseases or more in one case related to one systemic organ so, we have to evaluate all possible causes of current status of the patient.

Highlights

• •Xanthogranulomatous pyelonephritis is a distinct uncommon variant of chronic pyelonephritis.
• •Emphysematous pyelonephritis is also a rare condition.
• •The main cause of xanthogranulomatous pyelonephritis is Escherichia coli followed by proteus organisms.
• •The main treatment for both conditions is total nephrectomy.

1. Introduction

Pyelonephritis is defined as inflammation affecting the tubules, interstitium, and renal pelvis. It occurs in two forms. Acute pyelonephritis is generally caused by bacterial infection and is associated with urinary tract infection. Chronic pyelonephritis is a more complex disorder; bacterial infection plays a dominant role, but other factors (vesicoureteral reflux, obstruction) predispose to repeat episodes of acute pyelonephritis. The infecting organisms are derived from the patient's own fecal flora. By far the most common is Escherichia coli, followed by Proteus, Klebsiella, and Enterobacter[1]. Xanthogranulomatous pyelonephritis is an uncommon and distinct type of chronic infectious pyelonephritis in which yellow, lobulated masses diffusely replace the renal architecture.It is more common in adults in the fifth through the seventh decades. It is twice as common in women as in men. Urinary obstruction is almost invariably present and is most often caused by stones [2]. Emphysematous pyelonephritis has been defined as necrotizing infection of the renal parenchyma and its surrounding areas that result in the presence of gaz in the renal parenchyma, collecting system or perinephric tissue [3]. (see Fig. 1)

Fig. 1.

Microscopic picture of granulomas of foamy macrophages and gaz formation (empty space)with neutrophils and hemorrhage.

2. Case report

A 63-year-old man was admitted to the emergency room with complaint of bad general status, and right abdominal pain for two months with fever and chills. He received antibiotic therapy like gentamycin, for two weeks in venous route without any improvement.He had clinical history of diabetes mellitus and radical cystectomy since ten years. He treated diabetes mellitus with metformine 1000mg. He did radical cystectomy because of multiple stones found in it. At physical examination,he was conscious,blood pressure 11/7 mmHg (N = 12/8),pulse 85 bpm(N = 60–100), respiratory rate 28 rpm(N = 12–30),temperature 38,5 C(N = 37 C). Laboratory tests showed Red Blood Cells 2,65 (4-5millions), Haemoglobin 8,9 (N = 14–18), White blood Cells 15,46 (4000–10000),Platelets 446 (N = 150–450)with creatinine 1,8 (0,8-1,2), urea 77 mg/dl (N = 12–40),glucose 358mg/dl (N = 60–100), CRP 33 unit/ml.Urine culture was positive for klebsiella.Abdominal ultrasound revealed enlargement of right kidney with moderate hydronephrosis and calculus measures 2 cm in renal pelvis. computed tomography showed voluminous right kidney measures (13 × 13) cm with severe hydronephrosis and cortical thinning, air content, and oval large aggregates of turbid fluid with bullous gaz formations. he was submitted to right total nephrectomy (see Fig. 2).

Fig. 2.

Microscopic picture of granuloma of foamy macrophages and gaz formation (empty space).

3. Discussion

This study has points of strength that are available pathologic information, and limitation which are insufficient radiologic imaging. This work has been reported in line with SCARE 2020 criteria [4]. This case was diagnosed by the corresponding author and operated by surgical crew in Al-mowassat university hospital at 2021 June. The relevant medical literature talked about the two conditions as separated entities. Here, we have the two entities combined in one patient. Also, in one kidney. The patient was male. The main causes that interpreter patient condition are radical cystectomy he had since ten years and diabetes mellitus too.Gross examination reveals Yellow nodular tumor masses replace the renal pyramids, suppurative inflammation and edema begins within pelvic mucosa and sinus fat resulting in pelvicaliceal ulceration and fat necrosis,and nodules may become confluent and eventually involve renal capsule, perinephric fat, and retroperitoneal tissue [5]. Figure(3).Microscopic examination reveals central nidus of necrotic debris and neutrophils (microabscesses) with admixture of inflammatory cells, including lymphocytes, plasma cells, and Surrounding sheets of lipid-laden macrophages with abundant clear cytoplasm (may resemble clear cell renal cell carcinoma). Multinucleated giant cells and spindled fibroblasts surrounding macrophages (may resemble sarcomatoid carcinoma) may be seen [5].Figure (1),(2).

Fig. 3.

Gross appearance of this case reveals diffuse fibrosis and abscess formations.

4. Conclusion

It is necessary to look for all available findings about multiple disease processes and study it not only the direct cause.

Ethical approval

No ethical approval was needed.

Sources of funding

There was no funding

Author contribution

Rihan: study design, writing the paper. Mariam: study design, writing the paper.

Registration of research studies

1. Name of the registry: Research Registry.

2. Unique Identifying number or registration ID: Research registry 7028

https://www.researchregistry.com/browse-the-registry#home/

Guarantor

Dr. Rihan Mhmed Ali.

Consent

No consent obtained as consent form was not applicable to our study. This is retrospective study, collecting data from file of patients.

Provenance and peer review

Not commissioned, externally peer reviewed.

Declaration of competing interest

All the authors declare that they have no conflicts of interest.

Appendix A. Supplementary data

The following is the Supplementary data to this article: