Cyclophosphamide in steroid-dependent nephrotic syndrome

In order to determine the long-term effects of cyclophosphamide (CPO) and to identify parameters associated with sustained remission, we retrospectively studied the data from 90 patients with steroid-dependent nephrotic syndrome (SDNS) who received a single course of oral cyclophosphamide (2 mg/kg/day for 10 to 12 weeks). The median follow-up period after CPO was 5.5 years (interquartile range 3.2–8.5). Sustained remission reached the cumulative rate of 57% at 1 year, 42% at 2 years, and 31% at 5 years. For the patients who relapsed, the median threshold dose of prednisone between CPO initiation and first relapse has significantly decreased (22.1 mg/kg/day versus 4.9 mg/kg/day, p < 0.001). No further immunosuppressive agent was required in 60% of all patients. Young age at CPO initiation was associated with a lower rate of sustained remission (p < 0.001). Age at diagnosis of nephrotic syndrome, gender, cumulative dose of CPO (in mg/kg), and level of steroid dependence at CPO initiation did not influence the outcome. The incidence of side effects was low. These findings suggest that despite the wide use of new immunosuppressive agents, a short course of CPO remains an effective second-line therapy in SDNS patients. Optimal efficiency was observed in children over 7.5 years.

...We read with great interest the articles by Azib et al. [1] and Zagury et al. [2] that recently appeared in Pediatric Nephrology on the long-term prognosis of cyclophosphamide (CPA) for children with steroid-dependent nephrotic syndrome (SDNS)...
...CPA is a classical drug that has been used since the 1970s for treating refractory nephrotic syndrome, such as SDNS, frequent relapsing nephrotic syndrome (FRNS), and steroid-resistant nephrotic syndrome (SRNS) [1, 2] . However, both recent USA guidelines, published in 2009, and the Cochrane Reviews no longer strongly recommend using CPA for SDNS or SRNS because of its relatively low efficacy, gonadal toxicity, and carcinogenicity [3, 4]. ...
...The authors of both articles mentioned above as well as authors of a number of previous studies have reported that if CPA is used to treat children with SDNS, a 2-year relapse-free survival of CPA is achieved in 25–42% of patients [1, 2, 5, 6]...
...We observed that six of the nine children (66%) achieved 2-year relapse-free survival (Table 1). This rate is much better than that reported for single therapy with CPA [1, 2, 5, 6]...

Keywords (6)

Cyclophosphamide in steroid-dependent nephrotic syndrome,10.1007/s00467-011-1830-0,Pediatric Nephrology,Sonia Azib,Marie Alice Macher,Theresa Kwon,Agn

Citation Context (1)

Shuichi Ito, et al. Cyclophosphamide followed by mizoribine as maintenance therapy against...

References (18)

A meta-analysis of cytotoxic treatment for frequently relapsing nephrotic syndrome in children (Citations: 56)

Long-term effects of immunosuppressants in steroid-dependent nephrotic syndrome (Citations: 11)

CYCLOPHOSPHAMIDE TREATMENT IN STEROID-SENSITIVE NEPHROTIC SYNDROME OF CHILDHOOD (Citations: 19)

Cyclophosphamide in treatment of minimal change nephrotic syndrome (Citations: 6)

Eight and 12 week courses of cyclophosphamide in nephrotic syndrome (Citations: 26)

Citations (1)

Cyclophosphamide followed by mizoribine as maintenance therapy against refractory steroid-dependent nephrotic syndrome


	The following links allow you to view full publications. These links are maintained by other sources not affiliated with Microsoft Academic Search.


	BibTex \| RIS \| RefWorks Download