Objective: The determination of the ultimate prognosis for patients wi
th IgA nephropathy diagnosed in childhood requires long-term follow-up
of identified patients. The purpose of this study was to obtain such
follow-up for patients from two centers where the disease has been dia
gnosed for more than 20 years. Methods: Clinical data at the apparent
onset of symptoms and renal histologic data were obtained for 103 pati
ents in whom IgA nephropathy was diagnosed before age 18 years. Clinic
al status at last follow-up was obtained from office records or from d
irect contact with the patient. Predicted kidney survival was determin
ed by the Kaplan-Meier method. Follow-up of more than 10 years from th
e time of biopsy was available for 40 of the patients. Results: Fourte
en of the patients have progressed to end-stage renal disease; three o
thers have progressive chronic renal insufficiency as defined by an es
timated creatinine clearance of less than 50 ml/min per 1.73 m(2). Sev
erity of the renal histologic findings and the degree of proteinuria a
t the time of biopsy were associated with poor outcome. For all patien
ts, predicted kidney survival from the time of apparent onset was 94%
at 5 years, 87% at 10 years, 82% at 15 years, and 70% at 20 years. Age
at clinical onset and gender were not associated with poor outcome, b
ut black race and severity of renal histologic findings were. Conclusi
on: With fellow-up into adulthood, the outcome for pediatric patients
with IgA nephropathy appears to be as serious as that reported in adul
t patients. Follow-up of a pediatric patient with persistent clinical
findings should be maintained after the patient's care is transferred
to a physician caring for adults.