Question

• 7 year old male presents with rash and joint pain and is diagnosed with Henoch-Schonlein Purpura.  There is no evidence of renal disease on presentation.  Would corticosteroid treatment on presentation prevent delayed renal disease?

Clinical Bottom Lines

1. Children diagnosed with Henoch-Schonlein Purpura (HSP) have 20 to 90% risk of renal disease.  About 56% of those children with renal disease develop signs and symptoms of renal disease a week or more after presentation.¹ 
2. Incidence of renal failure in HSP nephritis is 2 to 5%.
3. Corticosteroids at a dose of 1mg/kg/day for 14 days appear to substantially reduce the incidence of renal disease in children with HSP who present without evidence of renal involvement.¹  
4. In the group of children who received steroids, none developed renal disease.  In the group that did not receive steroids, 11.9% developed renal disease 2 to 6 weeks after acute episode.
5. Renal disease was defined as 2 of the following:  proteinuria>4mg/m² , hematuria>10 RBC/HPF, BP>2 standard deviations above normal for age, BUN>54 or creatine>0.8.
6. None of the children in this study developed persistent renal insufficiency or ESRD

Summary of Key Evidence

1. 221 Children presented with HSP.  34 were excluded because they had evidence of renal involvement on presentation (hematuria and/or proteinuria).  19 were excluded because of poor follow-up (no mention is made of the group to which they had been assigned).  84 children were alternatively assigned to receive corticosteroid therapy.  The other 84 children received no corticosteroids.¹ 
2. Both groups were followed for two to three years.  Follow up was every 2 months for the first year, every 3 months for the second year and every 4 months for the 3rd year.  At each visit patients were examined and a urinalysis and BUN/Cr were obtained.
3. The major outcome measure was the development of renal disease which was defined as above. 
4. In the group that did not receive steroids, 10 children (11.9%) developed renal disease 2 to 6 weeks after presentation.  Of the 12 children with renal disease, 8 had no symptoms one year after onset.  Two children had persistent micro hematuria.  Two children had persistent proteinuria.

Additional Comments

• Two other studies evaluated the role of steroids in the prevention of delayed renal disease in HSP.  Both studies were retrospective.  Corticosteroids had been given for the treatment of abdominal or joint pain.  One study found the incidence of renal disease to be 4.3% (1/23) in the group treated with steroids and 50% (5/10) in the group that was not treated with steroids.²  Another study found the incidence of renal disease to be equal in both groups; 20% (4/20) in the group who received steroids and 20% (6/30) in the group that did not receive steroids.⁵
• In this study, 67% (8/12) of the children with nephritis had no symptoms one year later.  However, children who appear to recover may have significant renal disease many years later.⁴
• Corticosteroids have been found to improve abdominal pain and joint pain in HSP but they do not decrease the time of the acute phase.  There is no proven benefit of corticosteroids in the treatment of established HSP nephritis.
• Mechanism of renal disease in HSP is largely unknown therefore it is difficult to postulate how corticosteroids would prevent renal involvement.
• The major risks of corticosteroid treatment in children with HSP is masking an acute abdomen or intusseception and GI bleed.
• A very large prospective study is needed to determine the effect of early corticosteroid treatment in preventing renal insufficiency and ESRD.

Citation

1. Mollica F, LiVolti S, Garozzo R, Russo G: Effectiveness of early prednisone treatment in preventing the development of nephropathy in anaphylactoid purpura.  European Journal of Pediatrics.  151:140-144, 1992.
2. Buchanec J, Galanda V, Belakova S, Minarik M, Zibolen M: Incidence of renal complications in Henoch-Schonlein Purpura Syndrome in Dependence of an early administration of steroids.  International Urology and Neophrology.  20(4):409-412, 1988.
3. Bunchman TE, Mauer SM, Sibley RK, Verier RL: Anaphylactiod purpura: characteristics of 16 patients who progressed to renal failure.  Pediatric Nephrology.  2:393-397, 1988.
4. Goldstein AR, White RHR, Akuse R, Chantler C: Long term follow-up of childhood Henoch-Schonlein nephritis.  The Lancet.  339:280282, 1992.
5. Saulsbury F, Corticosteroid therapy does not prevent nephritis in Henoch-Schonlein Purpura.  Pediatric Nephrology.  7:69-71, 1993.

CAT Author: Katherine Layton, MD

CAT Appraisers: John Frohna, MD

Date appraised: October 12, 1998