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Balkan endemic nephropathy and associated urothelial cancer

Nature Clinical Practice Urology volume 5, pages 105–112 (2008)Cite this article

Abstract

Balkan endemic nephropathy (BEN) is a chronic tubulointerstitial disease described only in some rural parts of southeastern Europe. One of its most peculiar characteristics is a strong association with upper urothelial cancer (UUC). BEN-related UUC has the same histological features as other forms of UUC in general, but is more frequently bilateral, less frequently affects the bladder and has a sex ratio close to 1. BEN and BEN-associated UUC share the same etiology. Over time, incidence of these conditions has been declining. Since BEN was first described, around half a century ago, socioeconomic changes (in housing, farming, living standards, etc.) have been profound and have obscured the factors responsible for the observed reduction in incidence. Whatever the causes of BEN, the disease might not be restricted only to southeastern Europe. Rather, the intensity of exposure to risk factors for BEN and, consequently, clustering of cases has more likely determined our knowledge of topographical distribution of an etiological entity that is much more widespread, or that might even be ubiquitous in its sporadic form.

Key Points

  • Balkan endemic nephropathy (BEN) is an environmentally induced chronic tubulointerstitial kidney disease arising in areas around the tributaries of the River Danube in Bosnia, Bulgaria, Croatia, Romania and Serbia

  • The most recognizable feature of BEN is the clustering of cases in certain settlements

  • BEN is highly associated with transitional-cell upper urothelial cancer (UUC), both in BEN patients and in their family members

  • BEN-related UUC cases have some peculiarities that differentiate them from UUC cases in the rest of the world

  • Incidences of both BEN and BEN-related UUC have been decreasing over time; since intensity of mass exposure to the unknown causative agents is apparently diminishing, clustering of cases might fade away

  • BEN may well exist in the rest of the world in a sporadic form, as an undistinguishable part of the overall burden of tubulointerstitial nephropathies

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Figure 1: Topographical distribution of Balkan endemic nephropathy geographical foci.

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Authors and Affiliations

  1. V Stefanovic is Professor of Medicine at the Faculty of Medicine, University of Niš, Serbia.,

    Vladisav Stefanovic

  2. Z Radovanovic is Professor of Epidemiology, Faculty of Medicine, Institute of Nephrology, Kuwait.,

    Zoran Radovanovic

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  1. Vladisav Stefanovic
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  2. Zoran Radovanovic
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Correspondence to Vladisav Stefanovic.

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Stefanovic, V., Radovanovic, Z. Balkan endemic nephropathy and associated urothelial cancer. Nat Rev Urol 5, 105–112 (2008). https://doi.org/10.1038/ncpuro1019

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