Early high-dose immunosuppression in Henoch-Schönlein nephrotic syndrome may improve outcome.

Abstract

OBJECTIVE:

Renal involvement in Henoch-Schönlein purpura (HSP) constitutes a risk of end-stage renal disease (ESRD), especially in patients presenting with nephrotic syndrome.

PATIENTS AND 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.

CONCLUSION:

Follow-up data from the patients suggest that an early aggressive immunosuppressive approach improves long-term renal outcome in HSP patients with nephrotic syndrome.

• Authors: R F Andersen, S Rubak, B Jespersen, S Rittig
• Journal: Scandinavian journal of urology and nephrology (Scand J Urol Nephrol) Vol. 43 Issue 5 Pg. 409-15 ( 2009) ISSN: 1651-2065 [Electronic] England
• PMID: 19921987 (Publication Type: Comparative Study, Journal Article)

Chemical References

• Glucocorticoids
• Immunosuppressive Agents

Topics

• Adolescent
• Biopsy
• Child
• Dose-Response Relationship, Drug
• Drug Therapy, Combination
• Female
• Follow-Up Studies
• Glomerular Filtration Rate
• Glucocorticoids (administration & dosage)
• Humans
• IgA Vasculitis (complications, diagnosis, drug therapy)
• Immunosuppression Therapy (methods)
• Immunosuppressive Agents (administration & dosage)
• Male
• Nephrotic Syndrome (diagnosis, drug therapy, etiology)
• Retrospective Studies
• Time Factors
• Treatment Outcome