Long-term Outcome of Adult Onset Idiopathic Minimal Change Disease

Abstract

Minimal Change Disease (MCD) is the lesion most commonly associated with nephrotic syndrome in children, accounting for over 75% of cases. Although less common, MCD still accounts for up to 30% of adult onset nephrotic syndrome. Unlike children, in whom MCD is primarily idiopathic, secondary causes of MCD are seen in 13% of adults and must be considered, as the therapeutic approach to these patients is defined by the underlying cause. Clinical features at presentation in nephrotic adults with MCD can include microscopic hematuria, hypertension, and renal insufficiency, making MCD indistinguishable clinically from focal segmental glomerulosclerosis. As a result, a renal biopsy is required in adults in order to correctly diagnose and manage the nephrotic syndrome. As in children, response to therapy leads to a complete remission of proteinuria in up to 97% of adults, although, adults require a more prolonged course of therapy (16-28 weeks) compared to children (8 weeks). Relapse of MCD is extremely common in children (71%) and can be seen in up to 85% of adult patients. Relapses occur more frequently in younger adults (< 45 years) and are often seen in the first 6-12 months after the onset of a remission. Successful treatment of relapses can often be achieved with a second course of steroids. However, up to 50% of relapsing adults become frequent relapsers or steroid dependent. In these patients, a stable remission can be induced by treatment with either cyclophosphamide or cyclosporine. Overall, the long-term outcome of adult onset MCD is excellent, with fewer than 5% of patients progressing to end-stage renal disease and a patient survival of 83%-98% at 15 years.

Keywords

• Minimal change disease
• Nephrotic syndrome
• Proteinuria
• Treatment.