Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy
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CASE REPORT
Open Access
Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy Xiaoye Zhu1†, Lingxue Tu2†, Shaojun Liu1*, Huaizhou You1, Jun Xue1 and Chuanming Hao1
Abstract Background: Anti-low density lipoprotein receptor-related protein 2 (LRP2) nephropathy/anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies to kidney proximal tubular brush border protein LRP2/megalin. Patients are typically elderly and present with acute kidney injury and subnephrotic proteinuria. They progress to end-stage renal disease with poor response to immunosuppressive therapies. Case presentation: We report a case of a 29-year-old Chinese woman, who presented with nephrotic syndrome with normal kidney function. Kidney biopsy showed no obvious tubular injury or interstitial inflammation. Positive immunoglobulin G (IgG) staining was revealed along the brush border of proximal tubular cells. Anti-LRP2 antibody was identified in serum, consistent with a diagnosis of anti-LRP2 nephropathy. The patient achieved complete remission after receiving prednisone and cyclophosphamide. Conclusions: Anti-LRP2 nephropathy can also present as nephrotic syndrome in young patients and complete remission from nephrotic syndrome may be achieved after immunosuppressive therapy. Keywords: Anti-LRP2 nephropathy, Anti-brush border antibody, Kidney biopsy, LDL receptor-related protein 2, Renal pathology, Megalin
Background Anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies reactive to kidney proximal tubular brush border. ABBA was first described in 1981 and a total of 30 cases have been reported in the literature [1–8]. Circulating antibodies reactive to normal human kidney proximal tubular low-density lipoprotein (LDL) receptor-related protein 2 (LRP2), also known as * Correspondence: [email protected] † Xiaoye Zhu and Ling Xue Tu contributed equally to this work. 1 Division of Nephrology, Huashan Hospital, Fudan University, 12 middle Wulumuqi Road, Shanghai 200040, China Full list of author information is available at the end of the article
megalin, have been identified as being responsible for the disorder [4]. The largest cohort study, by Larsen et al, described 27 ABBA patients (mean age 70, range [49–90] years) who presented with acute kidney injury (AKI) (mean creatine 3.6 mg/dL, range [1.3–8.8] mg/dL) and subnephrotic range proteinuria [8]. Kidney biopsies demonstrated acute tubular injury with variable but mild tubulointerstitial inflammation. Tubular basement membrane (TBM) stained positive for immunoglobulin G (IgG) in all cases and LRP2 antigen was found to be colocalized with IgG along the TBM. Some of the patients had positive staining along the apical border of the proximal tubules. Most of the patients also had segmental
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