Chronic Kidney Disease of Unknown Etiology

A kidney disease of unknown cause is affecting up to 20% of men living in agricultural communities recognized to be hotspots, among them Nicaragua, El Salvador, Guatemala, and Sri Lanka. The disease often manifests in the third or fourth decade of life. Kidney biopsy indicates that the major finding is chronic tubulointerstitial nephritis in a majority of patients, although a subset may also present with symptoms of dysuria or fever and have acute interstitial nephritis on histology. In this chapter we review the epidemiology, pathology, and prominent hypotheses regarding potential causes of this mysterious kidney disease. We also provide a brief overview of ongoing population and occupational cohorts conducting promising investigations and/or interventions to prevent the disease.