Diagnostic Criteria for Balkan Endemic Nephropathy: Proposal by an International Panel

Figures & data

Table 1 Epidemiological, clinical and functional characteristics of BEN

Epidemiological characteristics
• Residence in an endemic settlement
• Family history of renal disease and of renal deaths
• Urothelial tumors
• Occupational history of farming
Clinical characteristics
• Slowly progressive renal insufficiency
• Anemia: normochromic or slightly hypochromic
• Edema absent
• Hypertension: rare in early, common in advanced renal failure
• Urothelial tumors: common
• Abnormalities on urinalysis
• Reduced kidney size, normal size in early stages
Functional changes
• Impaired concentrating capacity
• Decreased glomerular filtration rate
• Impaired urinary acidification
• Glycosuria, aminoaciduria
• Increased uric acid excretion
• Renal salt wasting
• Proteinuria of the tubular type

Modified from Stefanovic (1983).

Table 2 Clinical features and kidney morphology in BEN, analgesic nephropathy, and aristolochic acid nephropathy

	Balkan endemic nephropathy	Aristolochic acid nephropathy	Analgesic nephropathy
Familial character	Important feature	No	No
Associated urothelial cancer	Yes	Yes	Yes
Rate of progression	Very slow	Rapidly progressive	Slow
	(>20 years)	(6 months–2 years)	(>5 years)
Kidney imaging
Kidney size	Shrunken kidneys	Shrunken kidneys	Shrunken kidneys
Outline	Smooth surface	Smooth surface	Irregular contours
Calcifications	No	No	Medullary and papillary
Urinary deposit	Scarce deposit	Aseptic leukocyturia	Leukocyturia, microhematuria
Gross morphology
Kidney atrophy	Symmetric	Symmetric	Asymmetric
Outline	Smooth	Smooth	Irregular
Kidney histology
Tubular atrophy	3, Mainly outer cortex	3, Mainly outer cortex	3, Multifocal
Interstitial fibrosis	2, Mainly outer cortex	3, Mainly outer cortex	2, Multifocal
Interstitial cellular infiltration	1	1	2
Papillary necrosis	Absent	Absent	Hallmark
Etiology	Unknown	AA	Analgesics
Corticosteroid treatment	Not described	Slowed decrease of GFR	Not described

* Graded from 1 (present) to 3 (extensive).