24 research outputs found
Long-Term Outcome of Anti-Glomerular Basement Membrane Antibody Disease Treated with Immunoadsorption
<div><p>Background</p><p>Anti-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.</p><p>Objective</p><p>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.</p><p>Design</p><p>Retrospective review of patients treated with IAS for anti-GBM-antibody disease confirmed by biopsy and/or anti-GBM-antibodies.</p><p>Setting</p><p>University Hospital of Vienna, Austria.</p><p>Participants</p><p>10 patients with anti-GBM-disease treated with IAS.</p><p>Measurements</p><p>Patient and renal survival, renal histology, anti-GBM-antibodies.</p><p>Results</p><p>Anti-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.</p><p>Conclusion</p><p>IAS 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.</p></div
Cumulative costs of immunoadsorption and plasma exchange.
<p>Circles and triangles depict earliest timepoints of adsorber change due to reduced adsorption capacity (every 25 treatments for Immunosorba, every 35 treatments for Globaffin).</p
Extracorporal treatment and concomitant immunosuppression.
<p>Extracorporal treatment and concomitant immunosuppression.</p
Dynamics of serum-creatinine of individual patients.
<p>Observation period from start of IAS until 5 years. White coloured time points depict positive anti-GBM antibody testing.</p
Anti-GBM antibody levels from start of immunoadsorption until end of observation.
<p>Mean values ± standard deviation of all patients measured with ELISA depicted. Grey area denotes negativity of the assay.</p
Baseline characteristics of patients treated.
<p>Baseline characteristics of patients treated.</p
Absolute count and relative percentage of Dendritic cell subtype in histological sections.
<p>Absolute count and relative percentage of Dendritic cell subtype in histological sections.</p
CD141 positive cells in human kidney.
<p>Two distinct populations of CD11c<sup>+</sup> CD11b<sup>-</sup> (upper panels) and CD11c<sup>-</sup> CD11b<sup>+</sup> (lower panels) express the cDC1 marker CD141. CD11c<sup>+</sup> CD141<sup>+</sup> cells have the typical dendritic cell shape and high expression of HLA-DR but are negative for DC-SIGN. These characteristics identify them as cDC1 cells. The CD11b<sup>+</sup> CD141<sup>+</sup> have similar granularity to macrophages and express DC-SIGN together with HLA-DR. In here the plots of the kidney n° 5 are shown, similar results were obtained with the kidney n° 4. (b) In here the CD141 positive cells, plots of the kidney n°2 characterized by high numbers of infiltrating pDC are shown similar results were obtained with the kidneys n° 1 and 3.</p
Anti-GBM antibody kinetics and renal function in patient #7 (2a) and patient #10 (2b).
<p>Antibody values at diagnosis, before and after IAS, 12 hours in between sessions and up to 6 weeks; arrows depict daily IAS sessions. Grey area depicts negativity of the assay.</p