Abstract

BackgroundAnti-glomerular basement membrane (GBM) antibody disease may lead to acute crescentic glomerulonephritis with poor renal prognosis. Current therapy favours plasma exchange (PE) for removal of pathogenic antibodies. Immunoadsorption (IAS) is superior to PE regarding efficiency of antibody-removal and safety. Apart from anecdotal data, there is no systemic analysis of the long-term effects of IAS on anti-GBM-disease and antibody kinetics.ObjectiveTo examine the long-term effect of high-frequency IAS combined with standard immunosuppression on patient and renal survival in patients with anti-GBM-disease and to quantify antibody removal and kinetics through IAS.DesignRetrospective review of patients treated with IAS for anti-GBM-antibody disease confirmed by biopsy and/or anti-GBM-antibodies.SettingUniversity Hospital of Vienna, Austria.Participants10 patients with anti-GBM-disease treated with IAS.MeasurementsPatient and renal survival, renal histology, anti-GBM-antibodies.ResultsAnti-GBM-antibodies were reduced by the first 9 IAS treatments (mean number of 23) to negative levels in all patients. Renal survival was 40% at diagnosis, 70% after the end of IAS, 63% after one year and 50% at the end of observation (mean 84 months, range 9 to 186). Dialysis dependency was successfully reversed in three of six patients. Patient survival was 90% at the end of observation.ConclusionIAS efficiently eliminates anti-GBM-antibodies suggesting non-inferiority to PE with regard to renal and patient survival. Hence IAS should be considered as a valuable treatment option for anti-GBM-disease, especially in patients presenting with a high percentage of crescents and dialysis dependency due to an unusual high proportion of responders.

Highlights

  • Anti-glomerular basement membrane (GBM) disease is defined by circulating autoantibodies specific for the alpha-3 chain of type IV collagen [1] and characterised by focal necrotizing glomerulonephritis with linear deposition of IgG along the GBM

  • IAS should be considered as a valuable treatment option for anti-GBM-disease, especially in patients presenting with a high percentage of crescents and dialysis dependency due to an unusual high proportion of responders

  • After two plasma exchange (PE) and refractory anti-GBM-antibodies, she was converted to IAS and received 12 sessions over 27 days resulting in negative antiGBM-antibodies after 2 treatments but renal function did not recover

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Summary

Objective

To examine the long-term effect of high-frequency IAS combined with standard immunosuppression on patient and renal survival in patients with anti-GBM-disease and to quantify antibody removal and kinetics through IAS. Design: Retrospective review of patients treated with IAS for anti-GBM-antibody disease confirmed by biopsy and/or antiGBM-antibodies. Participants: 10 patients with anti-GBM-disease treated with IAS. Measurements: Patient and renal survival, renal histology, anti-GBM-antibodies

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