Management approach to children with steroid-resistant nephrotic syndrome

CoQ10: coenzyme Q10; CNI: calcineurin inhibitor; ACEi: angiotensin-converting enzyme inhibitor; ARB: angiotensin II receptor blocker; CKD: chronic kidney disease; MMF: mycophenolate mofetil; SRNS: steroid-resistant nephrotic syndrome.

* UpToDate suggests that genetic testing be performed in all patients with SRNS to identify monogenic forms of SRNS that are unlikely to respond to additional immunosuppressive therapy.

¶ If results of genetic testing are not expected to be available within 2 weeks, we suggest initiating therapy with a CNI (eg, cyclosporine or tacrolimus) while awaiting the results. Once results are available, treatment should be adjusted according to the approach in the algorithm.

Δ Dashed arrow indicates that a trial of CNI may be considered for selected patients with monogenic SRNS because a small percentage of these individuals respond. The possible benefit must be balanced against potential adverse effects, including nephrotoxicity and immunosuppression. CNI should be avoided in individuals with congenital nephrotic syndrome. For considerations, refer to UpToDate content on SRNS.

◊ Either cyclosporine or tacrolimus is acceptable. The available evidence suggests that cyclosporine and tacrolimus have similar efficacy and side effects in this setting. If there is no response after 6 months, CNI therapy should be discontinued and the patient should be encouraged to participate in a clinical trial evaluating novel therapies.

§ Some experts suggest giving an ACEi or ARB in conjunction with CNI therapies as part of the initial treatment for SRNS since ACEi or ARB may reduce proteinuria and slow progression of CKD. Other experts prefer to wait to assess the response to CNI therapy since the risk of nephrotoxicity is increased when CNIs are used in combination with ACEis/ARB and because some patients may have complete remission with CNI therapy alone. If the patient does not have complete remission within 4 months, ACEi or ARB therapy should be initiated. For additional details, refer to UpToDate content on SRNS.