This protocol is for adults with primary FSGS presenting with nephrotic syndrome who have relative contraindications or intolerance to glucocorticoids, and whose initial calcineurin inhibitor therapy did not achieve the required clinical response.
The patient has primary FSGS with nephrotic syndrome. Full glucocorticoid therapy is not appropriate due to a relative contraindication — for example, obesity, uncontrolled diabetes, a psychiatric condition, or severe osteoporosis — or documented intolerance. Calcineurin inhibitor therapy was used as the alternative initial immunosuppressive approach.
Calcineurin inhibitor therapy (cyclosporine or tacrolimus) was used as alternative initial immunosuppression. The therapeutic goals of reducing proteinuria and achieving remission were not met. This protocol represents the next clinical step following that failure.
Following calcineurin inhibitor failure in this setting, the approach centres on referral to specialised centres for expert assessment — including possible rebiopsy — and consideration of alternative treatment pathways or enrolment in a clinical trial. A specific combination immunosuppressive approach may additionally be considered when other therapeutic options have been exhausted.
In adults with relative contraindications or intolerance to glucocorticoids, alternative immunosuppression with CNIs should be considered as the initial therapy in patients with primary FSGS (Figure 54).
Additionally, patients who are obese, have uncontrolled diabetes, psychiatric conditions, or severe osteoporosis may be deemed to have a relative contraindication to glucocorticoids.
Adults who have steroid-resistant primary FSGS with resistance to or intolerance of CNIs should be referred to specialized centers for consideration of rebiopsy, alternative treatment, or enrollment in a clinical trial (Figure 55).
In considering these issues, the KDIGO 2021 Work Group agreed that it would be more appropriate to remove the use of MMF and high-dose dexamethasone as a clinical recommendation and consider this as an alternative treatment possibility when other therapeutic options have failed.
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