What is the role of calcineurin inhibitors (CNIs) in the treatment of frequently relapsing nephrotic syndrome (FRNS)?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

Calcineurin inhibitors (eg, cyclosporin A [CSA] and tacrolimus [TAC]) are useful steroid-sparing agents. These agents can also be used in children who fail to respond to, or subsequently relapse after, treatment with CYP, or in children whose families object to the use of CYP. There appears to be no difference in efficacy at 1 year between CSA and CYP. [72]

CNIs have disadvantages: prolonged courses of treatment are needed, nephrotic syndrome tends to recur when treatment is stopped, and nephrotoxic injury may occur. Consideration should be given to kidney biopsy after prolonged treatment to monitor for CNI-induced nephrotoxicity and fibrosis.

CSA has traditionally been the CNI most frequently used. However, limited studies suggest that TAC may be as effective as CSA. A 2-year prospective, uncontrolled trial demonstrated no difference between CSA and TAC in relapse at 1 and 2 years in children with FRNS and SDNS. [74] A single-center, randomized controlled trial by Choudhry et al found that TAC (0.1-0.2 mg/kg/day) and CSA (5-6 mg/kg/day) had similar efficacy in inducing remission in patients with idiopathic SRNS at 6 months and 1 year when combined with alternate-day low-dose corticosteroids and enalapril. [75]

The use of TAC reduces the risk of gingival hyperplasia and hirsutism associated with CSA, although nephrotoxicity is a risk with TAC as well as CSA. [75] It is the author's practice to use TAC in order to avoid the adverse effects of CSA.


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