Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Diagrams / tables | Clinical features | Diagnosis | Laboratory | Radiology description | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Virtual slides | Electron microscopy description | Genetics | Videos | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Karimi S, Setty S. Chronic pyelonephritis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneychronicpyelo.html. Accessed January 11th, 2025.
Definition / general
- Diffuse, patchy tubulointerstitial inflammation and scarring accompanied by blunting of calyces and the renal pelvis
Essential features
- Blunted, deformed, atrophic calyces with scarring due to chronic injury
- Diffuse, patchy lymphoplasmacytic tubulointerstitial inflammation and fibrosis
- Atrophic renal tubules with thyroid type tubular atrophy and intraluminal colloid-like proteinaceous casts
- References: Kumar: Robbins Basic Pathology, 10th Edition, 2017, Urol Case Rep 2018;19:65, Emerg Radiol 2020;27:561
Terminology
- Chronic pyelonephritis
ICD coding
Epidemiology
- F > M
- Common in pediatric population with congenital anomalies and vesicoureteral reflux disease
- Most common site: upper pole of the kidney
- Reference: Porter: The Merck Manual of Diagnosis and Therapy, 20th Edition, 2018
Sites
- Renal parenchyma
Pathophysiology
- Reflux of urine and ascending infection into the renal pelvis
- Obstructive uropathy
- Vesicoureteral reflux
- Chronic inflammation and infection leading to deformed and atrophic calyces with fibrosis and scarring of the renal parenchyma
- Recurrent damage and scarring leads to renal insufficiency and end stage renal disease (ESRD)
- Complicated chronic pyelonephritis is associated with nephrolithiasis
- Most common bacterial organism associated with chronic pyelonephritis: Escherichia coli
- Reference: Am J Physiol Renal Physiol 2017;312:F43, Radiol Med 2021;126:505, Am J Kidney Dis 2016;68:e23, Nephrol Dial Transplant 2006;21:1423
Etiology
- Urinary tract obstruction
- Reflux nephropathy including congenital abnormalities
- Recurrent, untreated pyelonephritis
- Neurogenic bladder
- Long term urethral catheterization
- References: Kumar: Robbins Basic Pathology, 10th Edition, 2017, J Am Geriatr Soc 1994;42:1286
Diagrams / tables
Clinical features
- Nonspecific, generalized malaise and abdominal pain
- Proteinuria in the nephrotic range; if severe, extensive damage to the renal parenchyma
- Silent clinical course if unilateral; presents as hypertension
- Recurrent acute pyelonephritis with fever, abdominal / flank pain
- Involvement of both kidneys can result in hyperchloremic acidosis
- References: Niger Postgrad Med J 2020;27:37, Porter: The Merck Manual of Diagnosis and Therapy, 20th Edition, 2018
Diagnosis
- Helical CT, intravenous urography (IVU)
- CT scan: gold standard for imaging assessment of the severity of pyelonephritis
- Voiding cystourethrogram for neonates, pediatric population with congenital abnormalities
- References: Porter: The Merck Manual of Diagnosis and Therapy, 20th Edition, 2018, Kidney Int 1999;55:1486, Radiographics 2008;28:255
Laboratory
- Elevated blood urea nitrogen (BUN) and elevated serum creatinine
- Urinalysis: proteinuria, renal epithelial cells, granular casts, pyuria (acute on chronic, recurrent acute pyelonephritis)
Radiology description
- Focal, polar, coarse cortical scars, calyceal distortion and renal atrophy
- Hypertrophy of residual renal parenchyma, mimicking infarct on imaging
- Ureteral dilation if severe reflux disease is present
- Obstruction with calculi may be present in obstructive nephropathy
- References: Emerg Radiol 2020;27:561, Radiographics 2008;28:255
Prognostic factors
- Slow progression, > 2 decades before consequences of damage are observed
- Can lead to end stage renal disease
- Reference: Porter: The Merck Manual of Diagnosis and Therapy, 20th Edition, 2018
Case reports
- 22 year old woman with xanthogranulomatous pyelonephritis (Urol Case Rep 2018;19:65)
- 68 year old woman with a nonenhancing left renal sinus mass (J Med Case Rep 2009;3:9054)
- 69 year old woman with primary chronic pyelonephritis (Ann Short Reports 2019;2:1039)
- 73 year old woman with chronic pyelonephritis and renal pyelocalyceal squamous cell carcinoma (J Med Case Rep 2019;13:154)
Treatment
- Surgical intervention: correction of anatomic / congenital abnormalities resulting in reflux
- Medical management: antimicrobial therapy for treatment of recurrent, acute pyelonephritis
- Long term antibiotic prophylaxis with trimethoprim / sulfamethoxazole, trimethoprim, nitrofurantoin, among others
- In pediatric patients who develop end stage renal disease due to reflux, transplant is a treatment modality
- References: Tunis Med 2018;96:495, Porter: The Merck Manual of Diagnosis and Therapy, 20th Edition, 2018
Microscopic (histologic) description
- Patchy interstitial lymphoplasmacytic inflammation with occasional, focal neutrophilic infiltration
- Patchy, well demarcated scarring of the renal pelvis and calyces
- Abundant intraluminal Tamm-Horsfall protein casts
- Tubular atrophy with thyroid type tubular atrophy and interstitial and periglomerular fibrosis
- Secondary segmental glomerulosclerosis
- References: Niger Postgrad Med J 2020;27:37, Yale J Biol Med 1985;58:91, Am J Kidney Dis 2016;68:e23
Microscopic (histologic) images
Electron microscopy description
- No specific findings
Genetics
- Mutant allele 299Gly TLR4 polymorphism
- References: Wiad Lek 2017;70:47, Pediatr Res 2007;61:371, PLoS One 2010;5:e14223
Videos
Overview of chronic pyelonephritis
Histological features of chronic pyelonephritis
Sample pathology report
- Kidney, radical nephrectomy:
- Renal cortical thinning with blunted calyces and scarring
- Global glomerulosclerosis with marked tubular atrophy and thyroid type tubular atrophy of the renal tubules
- Diffuse interstitial lymphoplasmacytic infiltration
- These findings are consistent with chronic pyelonephritis
Differential diagnosis
- End stage renal disease due to other predisposing causes:
- Cortical interstitial fibrosis, tubular atrophy, global glomerulosclerosis and arterionephrosclerosis
- Additional features vary based on the predisposing cause
- Xanthogranulomatous pyelonephritis:
- Dilated pelvicalyceal system
- Can be associated with multinucleated giant cells
- Sheets of foamy macrophages admixed with inflammatory cell infiltrate
- Hypertensive / vascular injury related renal disease:
- Adherent renal capsule that does not come off easily
- Sclerotic glomeruli with ischemic changes
- Arteriolar hyalinosis, arterial myointimal hyperplasia and arterionephrosclerosis
- References: Indian J Nephrol 2019;29:111, Curr Opin Nephrol Hypertens 2008;17:266, Kumar: Robbins Basic Pathology, 10th Edition, 2017
Board review style question #1
A 79 year old man presented with benign prostatic hyperplasia, obstructive uropathy and lobular segmental hypertrophy of the left kidney. A nephrectomy is performed revealing the histology above. What is the most likely diagnosis?
- Acute pyelonephritis
- Chronic pyelonephritis
- Diabetic nephropathy
- Emphysematous pyelonephritis
- Xanthogranulomatous pyelonephritis
Board review style answer #1
B. Chronic pyelonephritis. The image shown above illustrates atrophic tubules with luminal casts, diffuse interstitial lymphoplasmacytic infiltration, interstitial fibrosis and globally sclerosed glomeruli. The most likely histologic diagnosis is chronic pyelonephritis.
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Board review style question #2
Which of the following histologic features are associated with chronic pyelonephritis?
- Glomerular cellular crescents with segmental necrotizing change
- Histiocytes with granular eosinophilic cytoplasm and Michaelis-Guttman bodies
- Interstitial dense neutrophilic inflammation and tubular luminal neutrophils
- Tubular atrophy and luminal casts; interstitial lymphoplasmacytic inflammation and fibrosis
Board review style answer #2
D. Tubular atrophy and luminal casts; interstitial lymphoplasmacytic inflammation and fibrosis
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