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

• Main Authors: V. Thibaud, N. Rioux-Leclercq, C. Vigneau, S. Morice
• Format: Article
• Language: English
• Published: BMC 2019-01-01
• Series: BMC Nephrology
• Subjects: Kidney transplant; Goodpasture syndrome (GS); Anti-glomerular basement membrane (GBM) disease; End-stage renal disease (ESRD)
• Online Access: http://link.springer.com/article/10.1186/s12882-018-1197-6

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.