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

Treatment of Primary FSGS with Nephrotic Syndrome When Glucocorticoids Are Contraindicated or Not Tolerated

This protocol addresses adults with primary focal segmental glomerulosclerosis (FSGS) presenting with nephrotic syndrome who have a relative contraindication or intolerance to glucocorticoids — the conventional first-line therapeutic approach.

Clinical Scenario

In adults with primary FSGS and nephrotic syndrome, glucocorticoids may not be appropriate due to relative contraindications or intolerance. Patients who are obese, have uncontrolled diabetes, psychiatric conditions, or severe osteoporosis may each be deemed to have a relative contraindication to glucocorticoid therapy.

For these patients, alternative initial immunosuppression should be considered from the outset rather than proceeding with standard steroid-based treatment.

Treatment Approach (partial overview)

The evidence-based approach in this setting centres on a calcineurin inhibitor as alternative initial immunosuppressive therapy, used in place of glucocorticoids.

Agent selection, dosing targets, monitoring parameters, and the full management algorithm are detailed in the complete structured protocol below.

Treatment Goals

The primary clinical targets are reduction of proteinuria and achievement of remission.

Instant Access to Structured Evidence-Based Regimens
References
  1. 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).
  2. Additionally, patients who are obese, have uncontrolled diabetes, psychiatric conditions, or severe osteoporosis may be deemed to have a relative contraindication to glucocorticoids.
  3. Other observational studies looking at CNIs as first-line therapy for primary FSGS considered initial doses of cyclosporine at 3 mg/kg/d, with no therapeutic drug monitoring for a mean duration of 25 months, or tacrolimus at 4 mg/d with a target trough level of 4–7 ng/ml (5–9 nmol/l) for a mean duration of 13.6 ± 11.8 months.
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