Early high-dose immunosuppression in Henoch-Schönlein nephrotic syndrome may improve outcome.
OBJECTIVE
Renal involvement in Henoch-Schönlein purpura (HSP) constitutes a risk of end-stage renal disease (ESRD), especially in patients presenting with nephrotic syndrome.


METHODS
The clinical courses of six patients (mean age 13.2 years; four boys and two girls) admitted from 2000 to 2007 with HSP and nephrotic syndrome were reviewed. Average follow-up was 44 months (28-59). Treatment protocols included oral prednisolone and in non-responders cyclosporin A, cyclophosphamide, mycophenolate mofetil or tacrolimus. Five patients were treated immediately after presentation of nephrotic syndrome/nephrotic range proteinuria (median 277 mg/m(2)/h). The last patient was treated locally with low-dose prednisolone (0.2-0.9 mg/kg/day) and 3 months of low-dose cyclophosphamide (1 mg/kg/day).


RESULTS
All five patients treated promptly with high-dose immunosuppressant had normal estimated glomerular filtration rate (eGFR) (median 159 ml/min/1.73 m(2)) at follow-up. One obtained complete remission, two had positive dipstick proteinuria and two needed angiotensin-converting enzyme inhibitors to stay normotensive. The patient receiving low-dose immunosuppression at onset progressed to ESRD 44 months later. At initial presentation eGFR, blood pressure, renal biopsy grading, proteinuric range and plasma albumin were similar in all patients.


CONCLUSIONS
Follow-up data from the patients suggest that an early aggressive immunosuppressive approach improves long-term renal outcome in HSP patients with nephrotic syndrome.
Andersen
R F
RF
Department of Paediatrics, Aarhus University Hospital, Skejby, Aarhus, Denmark. rfa@ki.au.dk
Rubak
S
S
Jespersen
B
B
Rittig
S
S
D000293:Adolescent; D001706:Biopsy; D002648:Child; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D007165:Immunosuppression Therapy / Q000379:methods*; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents