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Abstract: SA-PO866

Efficacy of ANCA Autoantibody Clearance by High-Dose Immunoglobulins Prior to Plasma Exchange in Severe Pulmonary Renal Syndrome

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Author

  • Tampe, Bjoern, Universitatsmedizin Gottingen, Gottingen, Niedersachsen, Germany
Introduction

Plasma exchange rapidly depletes pathogenic anti-neutrophil cytoplasmic autoantibodies (ANCAs) and is considered for induction therapy in severe ANCA-associated vasculitis. The aim of plasma exchange is to remove putative disease mediators from the circulation, such as toxic macromolecules and pathogenic ANCAs.

Case Description

To our knowledge, we here provide the first report of applying high-dose IVIGs prior to plasma exchange and assessment of ANCA autoantibody elimination in a patient with severe pulmonary renal syndrome due to ANCA associated vasculitis. After high-dose application of intravenous immunoglobulins (IVIGs) prior to plasma exchange treatment, efficacy of myeloperoxidase (MPO)-ANCA autoantibody elimination was substantially increased, associated with rapid clearance of MPO-ANCA autoantibodies. High-dose IVIGs resulted in marked reduction of MPO-ANCA autoantibody levels and did not directly affect autoantibody clearance by plasma exchange itself, as also confirmed by comparable MPO-ANCAs in the exchange fluid relative to serum levels. Moreover, measurements of serum creatinine and albuminuria confirmed that high-dose IVIGs were well tolerated and did not exacerbate kidney injury.

Discussion

We are aware that our observations require validation in larger study populations, that kinetics may have also been affected by previous induction therapy, pretreatment time of IVIGs or ANCA autoantibody subtypes, and that the exact mechanisms contributing to our observations require further investigation. Furthermore, the immunoassay is not fully accurate due to values higher than the manufacturer’s reference range. Nevertheless, this ANCA level-driven approach might contribute to novel therapeutical strategies to increase efficacy of pathogenic ANCA autoantibody clearance in severe AAV.