Clinical outcome of Schonlein-Henoch purpura nephritis in children

We studied the long-term outcome of 64 children with biopsy-proven Schonlei n-Henoch purpura (SHP) nephritis over 1-23 years of follow-up. Overall rena l survival 10 years after onset was 73%. Multivariate logistic regression a nalysis identified initial renal insufficiency (P=0.004), nephrotic syndrom e (P=0.037), and the severity of histological alterations, as defined by th e proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more th an 19 years showed glomerular damage after transient recovery. Eight childr en with crescentic nephritis associated with a rapidly progressive course a nd/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatm ent serum creatinine levels dropped in each patient from a mean of 2.3 to 1 .1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patie nts produced comparable responses. Four patients undergoing PE reached end- stage renal disease at 1.2.-3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2-6.1 mg/dl after 7-13.5 years), a nd 1 patient reached a normal glomerular filtration rate. Our experience su ggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears t o delay the progression in some patients with severe, rapidly progressive f orms of the disease.