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.
Bibliographical noteFunding Information:
VA reports grants from ADDMEDICA , outside the submitted work; AB reports consulting fees from Chemocentryx, from Merck/MSD and from Astra Zeneca, outside the submitted work; MB reports lecture fees from Boehringer Ingelheim , from Bristol-Myers Squibb , from Novartis, travel support from Hexal, lecture fees and travel support from Vifor Fresenius and lecture fee from Servier, outside the submitted work; CKC reports grants from GlaxoSmithKline, grants and consultancy fees from Retrophin, outside the submitted work; SF reports lecture fees from VIFOR Pharma , outside the submitted work; CK reports being funded by National Institute for Health Research (NIHR) as academic clinical fellow (ACF), during the conduct of the study. All the other authors declared no competing interests.
© 2020 The Author(s)
- Focal segmental glomerulosclerosis
- Long-term remission
- Minimal change disease
- Nephrotic syndrome