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Published on: June 23, 2015
Predictive value of repeat renal biopsies in children with nephrotic syndrome
Abstract:
In children who exhibit a frequently relapsing course of minimal change disease (MCD), treatment is often difficult and frustrating to the physician and the family since the goal of a sustained remission remains elusive. The progression of the disease is often unpredictable from its clinical presentation since the lesion of MCD may evolve into a more severe form, such as mesangial IgM nephropathy or focal segmental glomerulosclerosis (FSGS), without alteration in signs and symptoms. Alkylating agents such as cyclophosphamide, or immunosuppressives such as cyclosporine can induce a more sustained remission, but are fraught with inherent toxicity, which makes difficult the decision to use these drugs in patients with MCD. Over a 10-year period we studied 49 patients who had more than one renal biopsy. Repeat biopsies were performed either to delineate the morphological lesion prior to change in therapy, or to confirm suspected drug toxicity, which would necessitate discontinuation of therapy. A total of 83 repeat biopsies were performed in these 49 patients. Of the 49 patients, 25 had MCD, and in 21 of these the lesion evolved into either IgM nephropathy (n = 7) or FSGS (n = 14). Of patients with IgM nephropathy (n = 12), 50% evolved into FSGS. The clinical diagnosis made prior to the repeat biopsy did not confirm with the histological diagnosis in 43% of cases, and a change in therapy or cessation of therapy was carried out in 43 of 83 repeat biopsy instances. Since the complications were mild and the ability of clinical findings to accurately predict the histological lesion limited, we conclude that repeat renal biopsies are a useful tool to fashion optimal therapy in children with frequently relapsing nephrotic syndrome.
Insights
Repeat renal biopsies help guide treatment for children with frequently relapsing minimal change disease (MCD). Histological findings often differ from clinical diagnoses, making biopsies essential for optimal therapy and managing potential disease progression to focal segmental glomerulosclerosis (FSGS).
Area of Science:
- Pediatric Nephrology
- Renal Pathology
- Immunosuppressive Therapy
Background:
- Minimal change disease (MCD) in children often presents with a relapsing course, making sustained remission challenging.
- MCD can unpredictably progress to more severe conditions like IgM nephropathy or focal segmental glomerulosclerosis (FSGS) without clear clinical changes.
- Standard immunosuppressive treatments carry toxicity risks, complicating therapeutic decisions for MCD.
Purpose of the Study:
- To evaluate the utility of repeat renal biopsies in managing children with frequently relapsing nephrotic syndrome.
- To assess the accuracy of clinical diagnoses versus histological findings in predicting disease course and guiding therapy.
- To determine if repeat biopsies aid in optimizing treatment strategies and managing disease progression.
Main Methods:
- A 10-year retrospective study of 49 children with frequently relapsing nephrotic syndrome who underwent at least one repeat renal biopsy.
- A total of 83 repeat biopsies were analyzed to correlate histological findings with clinical presentation and treatment changes.
- Analysis included tracking disease evolution from MCD to IgM nephropathy or FSGS and evaluating discrepancies between clinical and histological diagnoses.
Main Results:
- In 25 children with MCD, 21 showed lesion evolution, with 7 progressing to IgM nephropathy and 14 to FSGS.
- Of patients with IgM nephropathy, 50% evolved into FSGS.
- Clinical diagnosis mismatched histological findings in 43% of cases, leading to therapy adjustments in 43 of 83 repeat biopsies.
Conclusions:
- Repeat renal biopsies are valuable in managing children with frequently relapsing nephrotic syndrome, especially when clinical predictions are unreliable.
- The study highlights the significant rate of histological progression in MCD, underscoring the need for accurate diagnosis via biopsy.
- Repeat biopsies facilitate tailored therapeutic adjustments, improving management despite mild complications associated with the procedure.
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