Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis

In primary systemic small vessel vasculitis autoantibodies are common and seem to play an important role in the pathogenesis. Autoantibodies in vasculitis are preferentially directed against components of the immune system or directly against components of the vessel wall. Plasmapheresis is often ap...

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Main Authors: Mårten Segelmark, Lars Björck
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-09-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2019.02165/full
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author Mårten Segelmark
Lars Björck
author_facet Mårten Segelmark
Lars Björck
author_sort Mårten Segelmark
collection DOAJ
description In primary systemic small vessel vasculitis autoantibodies are common and seem to play an important role in the pathogenesis. Autoantibodies in vasculitis are preferentially directed against components of the immune system or directly against components of the vessel wall. Plasmapheresis is often applied in emergency situationists when the function of vital organs is jeopardized, the level of clinical evidence to apply such therapy, however, varies between low and non-existing. Plasmapheresis is a blunt and unspecific instrument that requires several sessions to achieve a substantial reduction of autoantibody levels. IdeS and EndoS are two relatively recently discovered enzymes produced by S. pyogenes, that have a remarkable capacity to degrade and disarm IgG. They have shown positive results in several in vivo models of autoimmunity, and treatment with IdeS has successfully been used to inactivate HLA alloantibodies in patients undergoing renal transplantation. Both IdeS and EndoS have the potential to become precision tools to replace plasmapheresis in the treatment of vasculitic emergencies and a clinical trial of IdeS in anti-GBM vasculitis is now ongoing.
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spelling doaj.art-bcad5a1dcfdf42d49726aeeae6cd379c2022-12-22T01:57:24ZengFrontiers Media S.A.Frontiers in Immunology1664-32242019-09-011010.3389/fimmu.2019.02165478373Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel VasculitisMårten Segelmark0Lars Björck1Nephrology, Department of Clinical Sciences, Lund University, Lund, SwedenInfection Medicine, Department of Clinical Sciences, Lund University, Lund, SwedenIn primary systemic small vessel vasculitis autoantibodies are common and seem to play an important role in the pathogenesis. Autoantibodies in vasculitis are preferentially directed against components of the immune system or directly against components of the vessel wall. Plasmapheresis is often applied in emergency situationists when the function of vital organs is jeopardized, the level of clinical evidence to apply such therapy, however, varies between low and non-existing. Plasmapheresis is a blunt and unspecific instrument that requires several sessions to achieve a substantial reduction of autoantibody levels. IdeS and EndoS are two relatively recently discovered enzymes produced by S. pyogenes, that have a remarkable capacity to degrade and disarm IgG. They have shown positive results in several in vivo models of autoimmunity, and treatment with IdeS has successfully been used to inactivate HLA alloantibodies in patients undergoing renal transplantation. Both IdeS and EndoS have the potential to become precision tools to replace plasmapheresis in the treatment of vasculitic emergencies and a clinical trial of IdeS in anti-GBM vasculitis is now ongoing.https://www.frontiersin.org/article/10.3389/fimmu.2019.02165/fullvasculitisANCAStreptococcus pygenesanti-GBM antibody diseaseautoantibodies
spellingShingle Mårten Segelmark
Lars Björck
Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis
Frontiers in Immunology
vasculitis
ANCA
Streptococcus pygenes
anti-GBM antibody disease
autoantibodies
title Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis
title_full Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis
title_fullStr Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis
title_full_unstemmed Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis
title_short Streptococcal Enzymes as Precision Tools Against Pathogenic IgG Autoantibodies in Small Vessel Vasculitis
title_sort streptococcal enzymes as precision tools against pathogenic igg autoantibodies in small vessel vasculitis
topic vasculitis
ANCA
Streptococcus pygenes
anti-GBM antibody disease
autoantibodies
url https://www.frontiersin.org/article/10.3389/fimmu.2019.02165/full
work_keys_str_mv AT martensegelmark streptococcalenzymesasprecisiontoolsagainstpathogeniciggautoantibodiesinsmallvesselvasculitis
AT larsbjorck streptococcalenzymesasprecisiontoolsagainstpathogeniciggautoantibodiesinsmallvesselvasculitis