Original Article
Time for Initial Response to Steroids Is a Major Prognostic Factor in Idiopathic Nephrotic Syndrome

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Objective

To identify early prognostic factors for idiopathic nephrotic syndrome (INS) in childhood.

Study design

A retrospective analysis of 103 patients with INS at onset, all treated in a single center with the same induction protocol, was conducted. Minimum length of follow-up was 2 years; median length of follow-up was 43 months. Survival data were assessed with Cox-Mantel analysis. Predictive values were estimated with receiver operating characteristic curves.

Results

The median time of response to steroid therapy was 7 days. A significant association was found between the interval from onset of steroid therapy to remission and the risk of relapsing within 3 months after steroid therapy discontinuation (P < .0001). A similar association was found between the time to achieve remission and the risk of developing frequent relapsing or steroid-dependent nephrotic syndrome (P < .0001), the prescription of maintenance steroid therapy (P < .003), and the prescription of all other non-steroid drugs (P < .0001) during follow-up. Patients with non-relapsing and infrequent relapsing nephrotic syndrome had a median time to achieve remission <7 days; in patients with frequent relapsing and steroid-dependent nephrotic syndrome, this median was >7 days.

Conclusion

The interval from onset of steroid therapy to remission is an accurate early prognostic factor in INS.

Section snippets

Methods

A retrospective IRB-approved analysis was performed on all patients with INS who were seen at our pediatric nephrology unit between 1992 and 2006 and who met these criteria: (1) patients were followed without interruption since disease onset; all secondary referrals were excluded; (2) the induction treatment corresponded to the International Study of Kidney Disease in Children (ISKDC) protocol (see below) and was initiated at our institution; (3) complete remission was achieved without the need

Results

The sex ratio and age at onset of INS reflect known frequencies for INS (Table I). Median length of follow-up was 43 months (range, 24-165 months). As indicated, approximately two-thirds of patients had FRNS or SDNS, and 9 patients did not have a relapse after their first episode. No patient received methylprednisolone boluses to achieve complete remission.

The median time of response to steroid therapy was 7 days, with 97% of patients responding within 28 days (Figure 1, A). With Cox-Mantel

Discussion

To date, steroids remain the mainstay initial course in the treatment of INS.2 Standard steroid therapy achieves remission in most patients and permits definition of INS subtypes. Patients that are resistant to steroids have a worse prognosis and carry mutations in constitutive podocyte genes in a significant proportion of cases.10, 11

Classification of patients who are steroid sensitive in different subgroups is useful to define the long-term prognosis, because patients with FRNS and SDNS

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Cited by (0)

Supported by the E-RARE Project “PodoNet: EU Consortium for Clinical, Genetic and Experimental Research into Hereditary Diseases of the Podocyte.” The authors declare no conflicts of interest

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