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      Multiple recurrences of anti-glomerular basement membrane disease with variable antibody detection: can the laboratory be trusted?

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          Abstract

          Anti-glomerular basement membrane (GBM) disease is commonly a monophasic illness. We present the case of multiple recurrences of anti-GBM disease with varying serum anti-GBM antibody findings. A 33-year-old female tobacco user presenting with hematuria was diagnosed with anti-GBM disease by renal biopsy. Five years later, she presented with alveolar hemorrhage and positive anti-GBM antibody. She presented a third time with alveolar hemorrhage but undetectable anti-GBM antibody. With each occurrence, symptoms resolved with plasmapheresis, intravenous methylprednisone and oral cyclophosphamide. The relationship between anti-GBM antibody findings and disease presentation is complex. Clinicians should be aware of the possibility of seronegative anti-GBM disease.

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          Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression.

          Anti-glomerular basement membrane (GBM) antibody disease is an autoantibody-mediated disorder that usually presents as rapidly progressive glomerulonephritis, often with pulmonary hemorrhage (the Goodpasture syndrome). It is reported that patients with severe renal failure do not generally recover renal function. To examine the long-term outcome of severe anti-GBM antibody disease. Retrospective review of patients treated for confirmed anti-GBM antibody disease over 25 years. A tertiary referral center in the United Kingdom. 71 treated patients with anti-GBM antibody disease. All patients received plasma exchange, prednisolone, and cyclophosphamide. Patient and renal survival, renal histology, and antibody levels. Patients who presented with a creatinine concentration less than 500 micromol/L (5.7 mg/dL) (n = 19) had 100% patient survival and 95% renal survival at 1 year and 84% patient survival and 74% renal survival at last follow-up. In patients who presented with a creatinine concentration of 500 micromol/L or more (>/=5.7 mg/dL) (n = 13) but did not require immediate dialysis, patient and renal survival were 83% and 82% at 1 year and 62% and 69% at last follow-up. In patients who presented with dialysis-dependent renal failure (n = 39), patient and renal survival were 65% and 8% at 1 year and 36% and 5% at last follow-up. All patients who required immediate dialysis and had 100% crescents on renal biopsy remained dialysis dependent. Patients with the Goodpasture syndrome and severe renal failure should be considered for urgent immunosuppression therapy, including plasma exchange, to maximize the chance of renal recovery. Patients needing immediate dialysis are less likely to recover.
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            Anti-glomerular basement membrane disease.

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              Goodpasture's disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques.

              Goodpasture's disease is characterized by rapidly progressive glomerulonephritis, often accompanied by pulmonary hemorrhage, in association with deposition of antibodies in a linear pattern on the glomerular basement membrane (GBM). The diagnosis of Goodpasture's disease in patients with acute renal failure often relies on the use of immunoassays to detect circulating anti-GBM antibodies in serum samples. We describe three cases of Goodpasture's disease in which no circulating anti-GBM antibodies were detectable in serum by well-established enzyme-linked immunosorbent assay or Western blotting techniques. The diagnosis of Goodpasture's disease was confirmed by renal biopsy, with linear deposition of immunoglobulin along the GBM and crescentic glomerulonephritis. In addition, an alternative method of antibody detection using a highly sensitive biosensor system confirmed that circulating antibodies were present in sera from both patients tested. Because this technique is not routinely available for the detection of anti-GBM antibodies, we suggest that diagnosis always be confirmed with a renal biopsy, and despite negative serological test results using immunoassay, the diagnosis of Goodpasture's disease should still be considered in the correct clinical context. Copyright 2002 by the National Kidney Foundation, Inc.
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                Author and article information

                Journal
                Clin Kidney J
                Clin Kidney J
                ckj
                ndtplus
                Clinical Kidney Journal
                Oxford University Press
                2048-8505
                2048-8513
                October 2016
                17 May 2016
                17 May 2016
                : 9
                : 5
                : 657-660
                Affiliations
                Division of Nephrology, Department of Medicine, University of Wisconsin School of Medicine and Public Health , Madison, WI, USA
                Author notes
                Correspondence and offprint requests to: Patricia Liu; E-mail: pliu891@ 123456gmail.com
                Article
                sfw038
                10.1093/ckj/sfw038
                5036896
                cc9f80be-10c6-4a2e-8a8f-f7491eb45f7c
                © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 13 February 2016
                : 14 April 2016
                Categories
                Atypical Anti-Gbm Disease

                Nephrology
                autoantibodies,crescentic glomerulonephritis,glomerulonephritis,plasmapheresis,renal biopsy

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