Definitions for the levels of evidence (I–IV) can be found at the end of the "Major Recommendations" field.
Guidelines
Cyclosporin may be effective in preserving filtration function in patients with steroid-resistant focal segmental glomerulosclerosis (FSGS), in those with steroid dependence or in those who frequently relapse on conventional therapy. (Level II evidence)
Suggestions for Clinical Care
(Suggestions are based on Level III and IV evidence)
A number of open studies have shown that cyclosporin is able to induce complete and partial remission in both adults and children with steroid resistant FSGS and steroid-dependent FSGS. Partial or complete remission is most likely in steroid-dependent FSGS, while the response rate in steroid-resistant FSGS is variable, ranging between 20% and 70% in most studies.
Cyclosporin is also associated with significant toxicity, which means that use of this agent should be reasonably restricted to patients at high risk of end-stage kidney disease (ESRD), or in whom toxicity from steroid-dependence confers a greater danger than chronic cyclosporin therapy.
What Dose Should Be Used?
Optimal dosing and monitoring of cyclosporin has not been fully clarified. Most studies have used doses of approximately 5 mg/kg/day with a blood level of 100–200 mg/mL. (Level III evidence)
Should Steroids Also Be Used?
Most studies have also continued a low dose of steroids while using cyclosporin. There is anecdotal evidence that this approach may be more effective in achieving remission in children than cyclosporin alone.
Optimal Duration of Therapy
A minimum effective dose of cyclosporin should be continued for at least 2 years. (Level IV evidence) Relapse is common after tapering or discontinuing the drug. Patients who are in complete remission for more than 1 year on cyclosporin appear to be more likely to remain in remission if the cyclosporine is gradually tapered and discontinued, rather than stopped suddenly. (Level IV evidence, anecdotal reports)
Definitions:
Levels of Evidence
Level I: Evidence obtained from a systematic review of all relevant randomized controlled trials (RCTs)
Level II: Evidence obtained from at least one properly designed RCT
Level III: Evidence obtained from well-designed pseudo-randomized controlled trials (alternate allocation or some other method); comparative studies with concurrent controls and allocation not randomized, cohort studies, case-control studies, interrupted time series with a control group; comparative studies with historical control, two or more single arm studies, interrupted time series without a parallel control group
Level IV: Evidence obtained from case series, either post-test or pretest/post-test
