A Case of Frequently Relapsing Minimal-Change Nephrotic Syndrome with Steroid-Induced Psychiatric Syndrome Treated by Low-Dose, Short-Term Steroid Therapy in Combination with Cyclosporine

Abstract

Adults with minimal-change nephrotic syndrome (MCNS) generally receive oral prednisolone (PSL) at an initial dosage of 1.0 mg/kg/day for a minimum of 4 weeks, with 80% of patients achieving clinical remission. However, relapses are frequent, necessitating repeated treatment with high-dose PSL. Long-term treatment with high-dose steroids increases the risk of steroid toxicities, such as diabetes mellitus, gastric complications, infections, osteoporosis, and steroid-induced psychiatric syndrome (SIPS), which may compromise the patient's quality of life. Strategies are therefore needed to reduce the dosage and duration of steroid therapy for frequently relapsing MCNS (FRNS). Here, we suggest a new combination therapy of low-dose and Case Kondo et al.: Nephrotic Syndrome Treated with Minimal Steroid Therapy and Cyclosporine 2short-term steroid with cyclosporine (CsA). We encountered an adult patient who developed recurrence of FRNS with depression arising from SIPS and was treated using low-dose, shortterm PSL combined with CsA. He was successfully treated with PSL at an initial dosage of 0.3 mg/kg/day (20 mg/day) for just 2 weeks combined with CsA, allowing earlier induction of complete remission. We then promptly reduced the dose of PSL to below a physiological dosage (5 mg/day) over 3 weeks without relapse after episodes of SIPS and quickly resolved psychiatric symptoms. CsA in combination with PSL can reduce the initial dosage of PSL, shorten the time to remission, and easily maintain clinical remission. This protocol appears clinically useful and potentially applicable as a future treatment strategy for FRNS troubled by SIPS.

Role of cyclosporine (CsA) in immuno-dermatological conditions

“…It has been reported that CsA can be prescribed in the pediatric population for indications such as “childhood psoriasis, pediatric dermatoses, auto-immune hemolytic anemia (AIHA), and the relapsing nephrotic syndrome”. [ 63 64 ]…”

Relapse treatment with low-dose steroids in steroid-sensitive minimal change disease

“…On the other hand, the widely known side effects of steroids have led to the exploration of new strategies that minimize the dose of corticosteroids utilized for MCD treatment ( 13 ). To date, the most widely employed strategy to minimize the steroid dose at the initial flare is based on the combination of LDS with non-steroidal immunosuppressants such as mycophenolate mofetil ( 14 , 15 ), tacrolimus ( 16 ), cyclophosphamide ( 17 ), cyclosporin ( 18 ), and the recently proposed rituximab ( 19 , 20 ). Despite the recurrent disease profile of this podocythopathy, only a limited number of studies have evaluated steroid minimization strategies for relapse therapy.…”

CD80 Insights as Therapeutic Target in the Current and Future Treatment Options of Frequent-Relapse Minimal Change Disease

“…The combination of CsA and prednisolone can reduce the initial dosage of prednisolone, hence shortening the time to remission and easily maintaining it. This combination protocol indicated the effectiveness and usefulness and could potentially be a future treatment strategy for frequent-relapse nephrotic syndrome [ 57 ]. The most crucial drawback of CsA ( Table 1 ) could be the high relapse rate after withdrawal of the medication.…”