Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP)

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.