Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment.

AIM: To identify risk factors for a child with Henoch-Schönlein purpura (HSP) either to develop nephritis (HSPN) or to contract progressive course and to obtain the currently available evidence on the efficacy of treatment options in both preventing and treating the established renal disease.

METHOD: Review of the literature published over the last two decades.

RESULTS: Persistent or recurrent purpura, severe abdominal symptoms and an older age proved as the most significant risk factors for later HSPN. The risks of long-term renal impairment are the highest in children having at presentation nephritic/nephrotic syndrome and/or more than 50% of glomeruli occupied by large crescents or sclerosing lesions. Randomized controlled trials (RCT) do not support short course prednisone at presentation of HSP in preventing persistent renal disease. Many uncontrolled studies using various treatment regimens have reported outcomes considered better than expected. However, the data from RCTs are sparse and no treatment options for the established renal disease can be currently recommended based on RCTs.

CONCLUSION: Severity and/or duration of extrarenal HSP symptoms and an older age are the most significant risk factors for developing HSPN, whereas clinical and histological severity at HSPN onset are in general predictive of a long-term renal impairment. The existing evidence does not support of short course prednisone in preventing persistent renal disease. A well-designed RCTs are needed in children with moderately severe or rapidly progressive (crescentic) HSPN.