Henoch-Schönlein nephritis: prognostic features and the challenge of therapy

Abstract

Henoch-Schönlein purpura is an intriguing entity of uncertain etiology associated with circulating IaA immune complexes, enhanced spontaneous immunoglobulin production, and an apparent imbalance in T cell regulatory functions. The associated glomerulonephritis is highly variable in nature and may profoundly influence clinical outcome. Prognostic features are reviewed in an effort to identify high-risk patients. Age, non-renal manifestations, non-selectivity of proteinuria, and evidence of preceding streptococcal infection are relatively weak predictors of end-stage renal disease. The presenting clinical and histologic expressions of nephritis are more useful prognosticators, but neither are completely reliable. Patients with greater than 50% crescents, and those with nephrotic syndrome complicated by various combinations of hypertension, azotemia, oliguria, and/or hypoproteinemia, are at increased risk of renal failure and might benefit from therapeutic interventions. Experience with various modalities is reviewed. Faced with uncontrolled clinical data, it is unclear whether immunosuppressive agents or plasmapheresis offer a therapeutic advantage over oral corticosteroids alone.