Evidence-based assessment of treatment options for children with IgA nephropathies

Abstract

We present an evidence-based evaluation of published data on therapy for children with various presentations of the IgA nephropathies--idiopathic IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (HSPN). Particular attention has been paid to the outcome markers used in the studies reviewed, with the best evidence provided by markers highly associated with progressive renal failure. No treatment modality for either IgAN or HSPN in pediatric patients has been shown to be effective by a properly designed and administered randomized controlled trial (i.e., the highest level of evidence--level 1). Lower levels of evidence support the use of a variety of corticosteroid regimens, often in combination with other agents, although there are some conflicting studies in this area. No convincing evidence has been published to date to support the use of fish oil, angiotensin-converting enzyme inhibitors or tonsillectomy for the treatment of children with IgAN or HSPN. Well designed randomized controlled trials in children with the IgA nephropathies need to be undertaken.

Steroid Treatment for Severe Childhood IgA Nephropathy

“…Although in a clinical trial of progressive IgAN the ultimate end point is development of chronic renal insufficiency, most pediatric patients do not develop it during the 2-yr study period (22). Our previous trial (5) and experience also support this.…”

“…With regard to children, our previous study (5) is the only randomized, controlled trial so far to have demonstrated that treatment that includes corticosteroid for 2 yr early in the course of disease reduces immunologic renal injury and prevents any further increase of glomerular sclerosis. Up to now, however, it has been unknown whether corticosteroid alone is sufficient for treatment of IgAN in children, and there has been no reliable evidence for its effectiveness in this group of patients (22). The difference in the effectiveness between the two treatment regimens in our study was thought to be due to the total effect of azathioprine, warfarin, and dipyridamole.…”

“…To date, information concerning not only the effectiveness but also the safety of corticosteroid therapy over a long time course has been largely defective. It has been difficult to assess the results of treatment trials with these agents in terms of preservation of renal function, as a result in part of wide variations in the length of therapy and the dosing regimens used and also to the use of corticosteroids in combination with other drugs (22). Recently, however, some evidence has been obtained for the role of corticosteroids in the treatment of IgAN (2,22).…”

“…It has been difficult to assess the results of treatment trials with these agents in terms of preservation of renal function, as a result in part of wide variations in the length of therapy and the dosing regimens used and also to the use of corticosteroids in combination with other drugs (22). Recently, however, some evidence has been obtained for the role of corticosteroids in the treatment of IgAN (2,22). In adults with IgAN, an Italian prospective, randomized, controlled trial demonstrated that a 6-mo course of steroid treatment protected against deterioration of renal function with no notable adverse effects during follow-up (23).…”

Long-Term Results of a Randomized Controlled Trial in Childhood IgA Nephropathy

“…Because of the variable rate of progression to renal failure and the probable multifactorial pathogenesis of IgA nephropathy (11), it is desirable to evaluate the effectiveness of any IgA nephropathy treatment by a prospective controlled trial. However, although the ultimate endpoint in any clinical trial of progressive IgA nephropathy is the development of chronic renal insufficiency, most pediatric patients do not develop chronic renal insufficiency during the study period (12). Therefore, the data in this study provide us with unique and valuable information about IgA nephropathy in children.…”