[Nephrotic syndrome: Current understanding and future therapies]

Area of Science:

• Nephrology
• Internal Medicine
• Clinical Research

Background:

• Advances in understanding nephrotic syndrome pathogenesis for minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS).
• The 2021 KDIGO-Guidelines emphasize differentiating primary, secondary, and genetic causes, crucial for treatment selection.
• Current therapies involve glucocorticoids for remission, with alternatives like calcineurin inhibitors, mycophenolate mofetil, cyclophosphamide, and rituximab for steroid-sparing management.

Purpose of the Study:

• To review current understanding and therapeutic strategies for MCD and FSGS.
• To highlight the importance of accurate classification in guiding treatment decisions.
• To discuss emerging therapies and the role of clinical registries.

Main Methods:

• Review of recent basic and clinical research findings.
• Analysis of the 2021 KDIGO-Guidelines for nephrotic syndrome management.
• Discussion of pharmacological agents and clinical trial data.

Main Results:

• Glucocorticoids remain central for primary MCD/FSGS remission.
• Several adjunct therapies (calcineurin inhibitors, MMF, CYC, rituximab) are available for steroid-sparing treatment.
• SGLT-2 inhibitors show potential for proteinuria reduction in non-diabetic CKD patients, including those with MCD/FSGS.
• Sparsentan, a dual endothelin/angiotensin receptor blocker, is a promising future therapeutic agent.

Conclusions:

• Accurate classification of MCD/FSGS is paramount for effective therapy.
• A broader range of immunosuppressants and novel agents like SGLT-2 inhibitors and Sparsentan are expanding treatment options.
• Inclusion in clinical registries is recommended for optimized therapy, genetic testing, and advancing individualized medicine.