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Mirioglu, S.; Daniel-Fischer, L.; Berke, I.; Ahmad, S.H.; Bajema, I.M.; Bruchfeld, A.; ... ; Kronbichler, A. (2024)

Management of adult patients with podocytopathies: an update from the ERA Immunonephrology Working Group

Article / Letter to editor
The histopathological lesions, minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are entities without immune complex deposits which can cause podocyte injury, thus are frequently grouped under the umbrella of podocytopathies. Whether MCD and FSGS may represent a spectrum of the same disease remains a matter of conjecture. Both frequently require repeated high-dose glucocorticoid therapy with alternative immunosuppressive treatments reserved for relapsing or resistant cases and response rates are variable. There is an unmet need to identify patients who should receive immunosuppressive therapies as opposed to those who would benefit from supportive strategies. Therapeutic trials focusing on MCD are scarce, and the evidence used for the 2021 Kidney Disease: Improving Global Outcomes (KDIGO) guideline for the management of glomerular diseases largely stems from observational and pediatric trials. In FSGS, the differentiation between primary forms and those with...Show moreThe histopathological lesions, minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are entities without immune complex deposits which can cause podocyte injury, thus are frequently grouped under the umbrella of podocytopathies. Whether MCD and FSGS may represent a spectrum of the same disease remains a matter of conjecture. Both frequently require repeated high-dose glucocorticoid therapy with alternative immunosuppressive treatments reserved for relapsing or resistant cases and response rates are variable. There is an unmet need to identify patients who should receive immunosuppressive therapies as opposed to those who would benefit from supportive strategies. Therapeutic trials focusing on MCD are scarce, and the evidence used for the 2021 Kidney Disease: Improving Global Outcomes (KDIGO) guideline for the management of glomerular diseases largely stems from observational and pediatric trials. In FSGS, the differentiation between primary forms and those with underlying genetic variants or secondary forms further complicates trial design. This article provides a perspective of the Immunonephrology Working Group (IWG) of the European Renal Association (ERA) and discusses the KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases focusing on the management of MCD and primary forms of FSGS in the context of recently published evidence, with a special emphasis on the role of rituximab, cyclophosphamide, supportive treatment options and ongoing clinical trials in the field.10.1093/ndt/gfae025 Video Watch the video of this contribution at https://academic.oup.com/ndt/pages/author_videos gfae025Media1 6346336829112Show less
FSGS
guideline
KDIGO
MCD
podocytopathy
All authors
Mirioglu, S.; Daniel-Fischer, L.; Berke, I.; Ahmad, S.H.; Bajema, I.M.; Bruchfeld, A.; Fernandez-Juarez, G.M.; Floege, J.; Frangou, E.; Goumenos, D.; Griffith, M.; Moran, S.M.; Kooten, C. van; Steiger, S.; Stevens, K.; Turkmen, K.; Willcocks, L.C.; Kronbichler, A.
Date
2024-02-10
Journal
Nephrology Dialysis Transplantation
DOI
doi:10.1093/ndt/gfae025