Rituximab in adult minimal change disease and focal segmental glomerulosclerosis - What is known and what is still unknown?

Philipp Gauckler, Jae Il Shin, Federico Alberici, Vincent Audard, Annette Bruchfeld, Martin Busch, Chee Kay Cheung, Matija Crnogorac, Elisa Delbarba, Kathrin Eller, Stanislas Faguer, Kresimir Galesic, Siân Griffin, Zdenka Hrušková, Anushya Jeyabalan, Alexandre Karras, Catherine King, Harbir Singh Kohli, Rutger Maas, Gert Mayer, Sergey Moiseev, Masahiro Muto, Balazs Odler, Ruth J Pepper, Luis F Quintana, Jai Radhakrishnan, Raja Ramachandran, Alan D Salama, Mårten Segelmark, Vladimír Tesař, Jack Wetzels, Lisa Willcocks, Martin Windpessl, Ladan Zand, Reza Zonozi, Andreas Kronbichler
Autoimmunity Reviews 2020 September 14, : 102671
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.

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