Abstract
Primary forms of minimal change disease and focal segmental glomerulosclerosis are rare podocytopathies and clinically characterized by nephrotic syndrome. Glucocorticoids are the cornerstone of the initial immunosuppressive treatment in these two entities. Especially among adults with minimal change disease or focal segmental glomerulosclerosis, relapses, steroid dependence or resistance are common and necessitate re-initiation of steroids and other immunosuppressants. Effective steroid-sparing therapies and introduction of less toxic immunosuppressive agents are urgently needed to reduce undesirable side effects, in particular for patients whose disease course is complex. Rituximab, a B cell depleting monoclonal antibody, is increasingly used off-label in these circumstances, despite a low level of evidence for adult patients. Hence, critical questions concerning drug-safety, long-term efficacy and the optimal regimen for rituximab-treatment remain unanswered. Evidence in the form of large, multicenter studies and randomized controlled trials are urgently needed to overcome these limitations.
Highlights
Nephrotic syndrome (NS) describes a clinical condition with heavy proteinuria, hypoalbuminemia, edema, hyperlipidemia and other as sociated features such as coagulation disorders and acute kidney injury
In kidney biopsies of patients with Minimal change disease (MCD), STAT6 activation was increased, suggesting IL-4 exposure in these patients [38]. These ob servations suggest that B cells are involved in the pathogenesis of MCD and focal segmental glomer ulosclerosis (FSGS), possibly by production of cytokines, such as IL-4 [38]
Current treatment strategies are based on supportive, antiproteinuric measures and immunosuppressive agents such as steroids, alkylating agents and Calcineurin inhibitors (CNI)
Summary
Nephrotic syndrome (NS) describes a clinical condition with heavy proteinuria, hypoalbuminemia, edema, hyperlipidemia and other as sociated features such as coagulation disorders and acute kidney injury. MCD accounts for only 10%–15% of adult patients with idiopathic NS and is associated with a longer time to re mission under corticosteroid treatment and a higher risk of acute kidney injury [3,4]. Overall long-term prognosis for adults with MCD is still excellent with remission rates of 75%–90% and a low risk for end stage kidney disease (ESKD) < 5% [5]. In addition to adaptive glomerular changes, the term secondary FSGS encompasses various other conditions such as genetic, virus-associated or drug-induced forms, that often are difficult to distinguish from true idiopathic/pri mary FSGS and should be managed conservatively, aiming to control blood pressure and proteinuria [6,9]. Remission [13,14]
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