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Core Concepts in Parenchymal Kidney Disease pp 125–135Cite as

Anti-glomerular Basement Membrane Disease

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Abstract

Anti-GBM disease is a rare but potentially fatal cause of a pulmonary-renal syndrome, presenting with pulmonary hemorrhage and rapidly progressive glomerulonephritis due to an underlying synchronous crescentic glomerulonephritis. It requires prompt diagnosis and rapid initiation of immunosuppression to ensure the best clinical outcomes, as more advanced disease at presentation is often associated with irreversible renal failure. The disease is autoimmune with both cellular and humoral immunity directed towards the alpha3 chain of type IV collagen, located in the alveolar and glomerular basement membranes. It has a number of genetic predispositions, including a very strong HLA association with HLA DR15, but may be triggered by various environmental factors. Therapy is based on established immunosuppressive protocols, with adjunctive plasmapheresis for rapid removal of pathogenic antibodies. Disease rarely relapses due to reestablishment of regulatory T cell populations but can recur in transplanted kidneys if circulating antibodies persist.

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  1. UCL Centre for Nephrology, Royal Free Hospital, London, UK

    Alan D. Salama M.B.B.S., M.A., Ph.D., F.R.C.P.

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  1. Alan D. Salama M.B.B.S., M.A., Ph.D., F.R.C.P.
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Correspondence to Alan D. Salama M.B.B.S., M.A., Ph.D., F.R.C.P. .

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Editors and Affiliations

  1. Div. Nephrology & Hypertension, Mayo Clinic Dept. Internal Medicine, Rochester, Minnesota, USA

    Fernando C. Fervenza

  2. Renal Division, Brigham and Women's Hospital, Boston, Massachusetts, USA

    Julie Lin

  3. Mayo Clinic, Rochester, Minnesota, USA

    Sanjeev Sethi

  4. Renal Division, Brigham and Women's Hospital Harvard Medical School, Boston, Massachusetts, USA

    Ajay K. Singh

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Salama, A.D. (2014). Anti-glomerular Basement Membrane Disease. In: Fervenza, F., Lin, J., Sethi, S., Singh, A. (eds) Core Concepts in Parenchymal Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8166-9_9

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