Prediction of high-degree steroid dependency in pediatric idiopathic nephrotic syndrome

Abstract

Most patients with idiopathic nephrotic syndrome are steroid-responsive, but about 50% relapse and often become steroid-dependent and exposed to long-term steroid complications. The aim of this study was to determine predictive risk factors for steroid and/or cyclosporine A (CyA) dependence. In France, steroid responsiveness is defined as remission after 1 month of oral prednisone (60 mg/m(2) per day) and-in the case of persistent proteinuria on day 30-three methylprednisolone pulses (MPP; 1 g/1.73 m(2) on days 1, 3, and 5). Thirty-five steroid-responsive children, followed between 1999 and 2006, were included in this study. Median age at diagnosis was 4.9 years. All patients initially received prednisone 60 mg/m(2) per day. Twenty-four of the 35 patients were steroid-dependent, with 12 requiring MPP. Of the latter 12 patients, 83.3% were treated with CyA during follow-up; in comparison, only 16.7% of the patients who did not receive MPP required CyA during follow-up (chi-square test, P = 0.001). T risk for steroid dependence was 100% in our cohort if remission was achieved after day 20. Patients who need MPP are at high risk to require CyA to achieve disease control. By identifying these children, we could use adequate immunosuppressive drugs earlier and reduce morbidity related to steroids and multiple relapses.

Primary Focal Segmental Glomerulosclerosis, an Important Component of Childhood Nephrotic Syndrome: Therapeutic Options and Genetic Basis

“…There is equal gender distribution in the African-American child [12], in contrast to the previous data of male predominance in the Caucasian child with FSGS [1,2]. The current treatment of choice is to slow the rate of progression [51], by a combination of vitamin E [18], prednisone [1], angiotensin converting enzyme inhibitor or angiotensin receptor blocker [14]. In the non-responsive cases, the addition of cyclosporine is supported by results of controlled clinical trials [22].…”

“…The definition of what comprises a complete remission remains problematic; it is generally accepted that, if the child has not completely cleared the urine of protein for 3-5 consecutive days after four weeks of prednisone, remission is incomplete. Some authors consider that a patient with two or more relapses during steroid tapering or within a month thereafter constitutes steroid-dependency [14]. These patients are classified as frequent relapsing/steroid-dependent.…”

Intravenous methylprednisolone in idiopathic childhood nephrotic syndrome

“…We believe our practice of administering ivMP for 3-5 days following non-response to four weeks of oral prednisolone, which is similar to the policy adopted by a majority of French Paediatric Nephrologists [4,5], has a number of advantages. It will help achieve remission in those children who require a higher dose of steroid to enter remission and also in those where adherence with initial oral therapy has been poor.…”

“…While good data exist regarding long-term outcomes following the ISKDC and other standard oral prednisone and prednisolone regimens, there are currently little long-term outcome data following regimens where additional ivMP is administered. One recent French study has reported an increased prevalence of steroid dependency and requirement for ciclosporin in children administered three doses of ivMP following failure to respond to 30 days of oral prednisone compared with those who responded to this initial course of oral therapy [5].…”

A Study on the Characteristics Determining Response to Steroids and Relapses in Nephrotic Syndrome in Paediatric Patients

“…3 The risks of a child developing frequent relapses or becoming steroid-dependent are increased with the number of relapses in the first 6 months after initial treatment, younger age at the initial episode, in boys, prolonged time to first remission, haematuria in first episode, shorter time to first relapse and infection at first relapse. [4][5][6][7][8][9] The most consistent indicator for a frequently relapsing course is early relapse after initial treatment. Studies have not assessed whether the other factors are independent risk factors for predicting frequent relapses or steroid dependence.…”