title: Long-term follow-up after cyclophosphamide therapy in steroid-dependent nephrotic syndrome.
abstract: Cyclophosphamide (CP) has been used for over 40 years in patients with steroid-sensitive nephrotic syndrome (SSNS) presenting frequent relapses or steroid dependence (SD). We evaluated retrospectively and tried to identify parameters possibly associated with a prolonged and sustained remission (PSR+) ≥5 years in 108 children with steroid-dependent nephrotic syndrome (SDNS) treated with oral CP. Patients had a follow-up time ≥5 years and were divided into two groups according to achievement of PSR (+ and -). Gender, histological injury, cumulative doses of CP, age of onset of illness, and start of treatment and prednisone dose on the occasion of relapse were analyzed. The overall cumulative sustained remission for 5 and 10 years was 25 and 21.6%, respectively. The only factor that influenced a PSR was the degree of SD: the group PSR+ relapsed at prednisone dose of 0.96 ± 0.51 mg/kg vs. 1.29 ± 0.59 mg/kg in group PSR- (p = 0.01). Also, patients who relapsed in the presence of prednisone doses ≤1.4 mg/kg showed a cumulative sustained remission of 43, 35, and 32.7% at 2, 5, and 10 years, respectively, versus 22.5, 12.5, and 5% in those with prednisone >1.4 mg/kg (p = 0.001). Our findings suggest that patients with SDNS who relapse on prednisone dose >1.4 mg/kg are especially prone to an unfavorable response to CP use.
authors.affiliation: Department of Pediatric Nephrology, Hospital Federal de Bonsucesso, Av. Londres 616 - Bonsucesso, Rio de Janeiro, CEP: 21041-030, RJ, Brazil. alberto.zagury@gmail.com
authors.forename: Alberto
authors.initials: A
authors.lastname: Zagury
authors.forename: Anne Louise
authors.initials: AL
authors.lastname: de Oliveira
authors.forename: Carlos Augusto Pinheiro
authors.initials: CA
authors.lastname: de Moraes
authors.forename: Jose Augusto
authors.initials: JA
authors.lastname: de Araujo Montalvão
authors.forename: Regina Helena Leite Lemos
authors.initials: RH
authors.lastname: Novaes
authors.forename: Vinicius Martins
authors.initials: VM
authors.lastname: de Sá
authors.forename: Deise De Boni
authors.initials: Dde B
authors.lastname: Monteiro de Carvalho
authors.forename: Tereza
authors.initials: T
authors.lastname: Matuck
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use*; D013997:Time Factors
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide