Focal segmental glomerulosclerosis
ICD-10 N04.1 · ICD-11 MF8Y.2

Primary FSGS with Nephrotic Syndrome: Treatment After Glucocorticoid Failure

Clinical Scenario

This protocol addresses adults with primary FSGS presenting with nephrotic syndrome, who have received high-dose oral glucocorticoids as first-line immunosuppressive therapy but have not achieved complete remission.

When First-Line Treatment Is Insufficient

High-dose oral glucocorticoids are the recommended first-line immunosuppressive treatment for primary FSGS. The expected outcome is reduction of proteinuria and complete remission — typically apparent within weeks of starting therapy. When these targets are not reached and the patient is classified as steroid-resistant, escalation to a next-line approach is indicated.

Next-Line Approach (Partial Overview)

For adults with steroid-resistant primary FSGS and nephrotic syndrome, the protocol centres on a calcineurin inhibitor-based regimen. The complete treatment algorithm — including agent choice, duration, and monitoring parameters — is available in the full protocol.

Treatment Goals

The aim is remission and meaningful reduction of proteinuria. Responses may emerge gradually; in some patients, remission has been reported to take as long as 4–6 months.

Instant Access to Structured Evidence-Based Regimens

References

  1. We recommend that high-dose oral glucocorticoids be used as the first-line immunosuppressive treatment for primary FSGS (1D).
  2. For adults with steroid-resistant primary FSGS, we recommend that cyclosporine or tacrolimus be given for ‡6 months rather than continuing with glucocorticoid monotherapy or not treating (1C).
  3. Since remissions after the use of cyclosporine may occur slowly and have been reported to take as long as 4–6 months in certain observational studies, we suggest that a minimum treatment duration of 6 months should be attempted before labeling a patient as cyclosporine-resistant.
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