Progressive hereditary nephropathy is described in 6 members of a sing
le family. Renal biopsies, performed in 3 patients, revealed tubular a
trophy, interstitial fibrosis, and lymphomonocytic infiltration associ
ated with severe vascular lesions. These features were disproportionat
ely serious when related to age, arterial pressure, and renal function
. Similar familial nephropathy has been reported in the literature, ge
nerally in association with gout or asymptomatic hyperuricemia. The pa
tients described here had normal blood concentrations of uric acid. It
is proposed that the members of the present group of patients are suf
fering from the same interstitial nephropathy as that described in the
literature and that the hyperuricemia found by other investigators is
coincidental and does not play a pathogenetic role.