What is the prognosis of pediatric nephrotic syndrome responsive to steroids?

Updated: Mar 04, 2020
  • Author: Jerome C Lane, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

Patients who remain responsive to steroids with remission of proteinuria, even with frequent relapses, generally have a good prognosis. The ISKDC found that in 93% of children with INS who responded to steroids, kidney biopsy revealed MCNS. [5] In contrast, 75% of patients who did not initially respond to steroids had histology other than MCNS.

About 90% of children with MCNS (but only 20% of children with FSGS) achieve remission after the initial course of steroid treatment.

Despite the generally favorable prognosis in patients who respond to steroids, the ISKDC reported a 60% rate of subsequent relapses, which can lead to complications, increased morbidity, and decreased quality of life. [5] A longer course of initial steroid treatment (12 weeks rather than the original ISKDC protocol of 8 weeks) may reduce the rate of subsequent relapse to 36%, [48] which still represents a large number of patients who undergo repeated courses of immunosuppression, with possible hospitalizations, edema, infections, medication adverse effects, and other comorbidities.

A long-term study of 398 children with INS found that the percentage of children who became free of relapses during the course of their disease rose from 44% at 1 year after diagnosis to 69% at 5 years and 84% at 10 years after diagnosis. [41, 49] Although most children with INS who respond to steroids achieve long-term remission, relapses may continue into adulthood.

Older studies suggested that more than 90% of children achieve long-term remission without further relapses by puberty. However, this has been challenged by surveys indicating a rate of relapse during adulthood as high as 27-42%. [50]

In a retrospective study, Vivarelli et al reported that the length of time between initiation of steroid treatment and syndrome remission is an early prognostic indicator for children with INS. [51] In study participants who did not suffer relapse or who relapsed infrequently, the median time from treatment onset to remission was less than 7 days. In patients who had frequent relapses or who developed steroid-dependent nephrotic syndrome, the median time to remission was more than 7 days.

A study of 42 adult patients with a history of childhood INS found that 33% of patients continued to relapse into adulthood. Fortunately, overall morbidity (eg, bone disease, infections, malignancies, cardiovascular complications) remained low, and patients had normal adult height, body mass index (BMI), and kidney function. Predictors of adult relapse included the number of relapses during childhood and the use of immunosuppressant medications other than steroids (ie, cyclosporine, chlorambucil, cyclophosphamide). [52]


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