Rapidly progressive interstitial renal fibrosis has recently been repo
rted in young women who have been on a slimming regimen including Chin
ese herbs. We examined four nephroureterectomies performed in three pa
tients prior to or at the time of transplantation to determine the nat
ure and topography of the kidney and urinary tract lesions in Chinese
herbs nephropathy (CHN). Extensive, hypocellular, interstitial scleros
is, tubular atrophy and global sclerosis of glomeruli decreasing from
the outer to the inner cortex, including the columns of Bertin, were o
bserved in the four kidney specimens, together with severe fibromucoid
to fibrous intimal thickening, mainly of interlobular arteries, norma
l or collapsed residual glomeruli, and mild to moderate atypia and aty
pical hyperplasia of the urothelium. In addition, bilateral pelviurete
ric sclerosis was observed in one case. With the exception of the latt
er, these lesions are very similar to those described in Balkan endemi
c nephropathy (BEN). The clinical presentation of the patients was als
o similar to that observed in BEN: normal blood pressure, aseptic leuk
ocyturia, low grade low molecular weight proteinuria, early and severe
anemia. In conclusion, on morphological and clinical grounds, CHN app
ears similar to BEN. A common etiologic agent, aristolochic acid, is s
uspected. The known carcinogenic potential of this compound, taken tog
ether with our finding of multiple foci of cellular atypia of the urot
helium suggest that CHN patients should undergo a regular follow-up fo
r urothelial malignancy.