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Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome

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Abstract

Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children‘s Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide.

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Authors and Affiliations

  1. Renal Division, Department of Pediatrics, Baylor College of Medicine, 6621 Fannin St., MC3-2482, Houston, TX, 77030, USA

    Sarah J. Swartz & Daniel I. Feig

  2. Department of Pathology, Baylor College of Medicine, Houston, TX, USA

    Karen W. Eldin & M. John Hicks

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  1. Sarah J. Swartz
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  2. Karen W. Eldin
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  3. M. John Hicks
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  4. Daniel I. Feig
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Correspondence to Daniel I. Feig.

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Swartz, S.J., Eldin, K.W., Hicks, M.J. et al. Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome. Pediatr Nephrol 24, 1187–1192 (2009). https://doi.org/10.1007/s00467-009-1130-0

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  • DOI: https://doi.org/10.1007/s00467-009-1130-0

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