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Recurrence of Goodpasture syndrome without circulating anti-glomerular basement membrane antibodies after kidney transplant, a case report

Abstract : Background - Goodpasture Syndrome (GS) is an autoimmune disease caused by the development of auto-antibodies against the Glomerular Basement Membrane (GBM). Linear deposit of immunoglobulins G on the GBM detected by immunofluorescence analysis of renal biopsies is a GS pathognomonic finding. GS is commonly monophasic and its incidence is 1.6 case per million per year. Case presentation - This report describes and discusses the case of a 40-year-old woman who one year after allograft kidney transplant, presented with acute pulmonary and renal symptoms of GS, leading to acute graft dysfunction, without circulating anti-GBM antibody detection in laboratory assays. She received a living donor kidney transplant 4 years after the first diagnosis of GS without circulating anti-GBM antibodies, when considered in remission. Conclusions - In both episodes, the diagnosis of GS was based exclusively on the kidney biopsy that showed rapidly progressing glomerulonephritis with deposition of immunoglobulins G on the GBM. Although rare, the management of patients with GS without circulating anti-GBM antibodies is difficult due to the lack of standardized follow-up guidelines to reduce the risk of GS recurrence after kidney transplantation.
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https://hal-univ-rennes1.archives-ouvertes.fr/hal-02445337
Contributor : Laurent Jonchère <>
Submitted on : Thursday, July 9, 2020 - 10:43:05 AM
Last modification on : Monday, July 20, 2020 - 4:02:34 PM

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s12882-018-1197-6
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V. Thibaud, N. Rioux-Leclercq, C. Vigneau, S. Morice. Recurrence of Goodpasture syndrome without circulating anti-glomerular basement membrane antibodies after kidney transplant, a case report. BMC Nephrology, BioMed Central, 2019, 20 (1), pp.6. ⟨10.1186/s12882-018-1197-6⟩. ⟨hal-02445337⟩

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