Abstract
There are various histopathological forms of idiopathic nephrotic syndrome, including minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). Whereas some relapse predictor factors have been identified in renal transplantation, the clinical future of idiopathic nephrotic syndrome in the native kidney remains uncertain. We designed a multicentric retrospective descriptive cohort study including all patients aged 15 years and over whose renal biopsy confirmed MCD or FSGS between January 2007 and December 2014. We studied 165 patients with idiopathic nephrotic syndrome; 97 with MCD and 68 with FSGS. In the MCD cohort, 91.7% of patients were treated with corticosteroids for a median total duration of 13 months. During 45 months of follow-up, 92.8% of patients achieved remission and 45.5% experienced relapse. In this cohort, 5% of patients experienced terminal kidney disease. With respect to FSGS patients, 51.5% were treated with corticosteroids for a median total duration of 15 months. During 66 months of follow-up, 73.5% of patients achieved remission and 20% experienced relapse. In this cohort, 26.5% of patients experienced terminal kidney disease. No statistical association was observed between clinical and biological initial presentation and relapse occurrence. This study describes the characteristics of a cohort of patients with the nephrotic idiopathic syndromes of MCD and FSGS from the time of renal biopsy and throughout follow-up.
Highlights
Idiopathic nephrotic syndrome (INS) accounts for 15–30% of adult glomerulopathies [1]
We reported a complete description of a multicentric idiopathic minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) population
We report a French multicentric cohort of idiopathic minimal change disease and focal segmental glomerulosclerosis
Summary
Idiopathic nephrotic syndrome (INS) accounts for 15–30% of adult glomerulopathies [1]. The existence of circulating permeability factor(s) in the plasma of patients with idiopathic nephrotic syndrome was suggested a few years ago through different observations: resolution of FSGS after kidney transplantation from a donor with FSGS to a healthy recipient [2]; efficacy of plasmatic exchanges and/or immunoadsorption to obtain INS remission in native or transplanted kidney disease [3,4]; transient mother-to-fetus proteinuria transmission [5]; and murine models in which injection of T lymphocyte supernatant or serum from patients with INS induced proteinuria with histological MCD or FSGS lesions [6,7]. INS is associated to a cytokine environment leading to abnormal T-cell response and abnormal cooperation between T- and B-cells [14]
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