Cochrane review: Interventions for preventing and treating kidney disease in Henoch‐Schönlein Purpura (HSP)
Authors: Chartapisak Wattana , Opastirakul Sauwalak , Hodson Elisabeth M , Willis Narelle S , Craig Jonathan C 
Published: September 2010

Background

To determine the benefits and harms of therapies used to prevent or treat kidney disease in Henoch‐Schönlein Purpura (HSP).

Objectives

To evaluate the benefits and harms of different agents (used singularly or in combination) compared with placebo or no treatment or another agent for the prevention or treatment of kidney disease in patients with HSP.

Search strategy

Randomised controlled trials (RCTs) and quasi‐RCTs were identified from the Cochrane Renal Group's specialised register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE using optimally sensitive search strategies combined with search terms for HSP.

Selection criteria

RCTs comparing any intervention used to prevent or treat kidney disease in HSP compared with placebo, no treatment or other agents were included.

Data collection and analysis

Three authors independently assessed trial quality and extracted data from each study. Statistical analyses were performed using the random effects model and the results were expressed as risk ratio (RR) for dichotomous outcomes and mean difference (MD) for continuous outcomes with 95% confidence intervals (CI).

Main results

Ten studies (1230 children) were identified. There was no significant difference in the risk of persistent kidney disease at six months (3 studies, 379 children: RR 0.51, 95% CI 0.24 to 1.11) and 12 months (3 studies, 498 children: RR 1.02, 95% CI 0.40 to 2.62) in children given prednisone for 14 to 28 days at presentation of HSP compared with placebo or supportive treatment. In children with severe kidney disease, there was no significant difference in the risk of persistent kidney disease with cyclophosphamide compared with supportive treatment (1 study, 56 children: RR 1.07, 95% CI 0.65 to 1.78) and with cyclosporin compared with methylprednisolone (1 study, 19 children: RR 0.39, 95% CI 0.14 to 1.06).

Authors' conclusions

Data from RCTs for any intervention used in improve kidney outcomes in children with HSP are very sparse except for short‐term prednisone. There was no evidence of benefit of prednisone in preventing serious long‐term kidney disease in HSP.

Plain Language Summary

Interventions to prevent or treat serious kidney disease in patients with Henoch‐Schönlein Purpura

Henoch‐Schönlein Purpura (HSP) causes inflammation of small blood vessels in children and affects approximately 20/100,000 children annually. Symptoms and signs include a purpuric skin rash (which comprises small spots and larger bruises), abdominal pain, gastrointestinal bleeding, joint pain and swelling, facial swelling and evidence of kidney disease with blood and protein in the urine. Kidney disease occurs in about one third of children with HSP. In the majority this is mild (small amounts of blood in the urine only) and resolves completely but a few children have persistent kidney disease that can progress to kidney failure. Treatments with medications that suppress the immune system (prednisone) and treatments to prevent blood clotting (aspirin, heparin) have been administered to children at diagnosis to prevent serious kidney disease. Also treatments which suppress the immune system (prednisone, methylprednisolone, cyclophosphamide and cyclosporin) have been used in an attempt to treat serious kidney disease in HSP and prevent progression to kidney failure.

This review identified five studies (789 children) which compared prednisone tablets given for 14‐28 days with placebo tablets or no specific treatment for the prevention of serious kidney disease at 6‐12 months after onset of HSP. No significant reduction in the frequency of serious kidney disease was demonstrated. Two studies (138 children) showed no benefit of aspirin and dipyridamole (antiplatelet agents) to prevent serious kidney disease. One study (228 children) suggested that heparin given by injection could reduce the risk for serious kidney disease but this treatment has the potential side effect of severe bleeding so its administration is not justified when only one third of children develop kidney disease and in most this is not serious and resolves completely.

In children with serious kidney disease, one study (56 children) showed that cyclophosphamide was no more effective than supportive treatment in preventing kidney failure. A second study (19 children) found no difference in benefit between cyclosporin and methylprednisolone/prednisone but numbers were too small to exclude a benefit completely.

Most studies did not provide data on side effects of the treatments given.

There are few data from randomised studies examining interventions used to prevent or treat serious kidney disease in HSP except for short‐term prednisone to prevent kidney disease. There was no evidence of benefit of prednisone compared with placebo or no specific therapy in preventing serious kidney disease in HSP.