Current state of biologicals in the management of systemic vasculitis

Peter Lamprecht*, Andreas Till, Jörg Steinmann, Peer M. Aries, Wolfgang L. Gross

*Corresponding author for this work
14 Citations (Scopus)


Conventional immunosuppressive treatment of systemic vasculitides has improved their often fatal outcome, but is burdened by cytotoxic side effects and frequent relapses. Recent advances in the therapy of systemic vasculitides with biologicals have helped to establish new options for patients resistant to conventional treatment. Moreover, early intervention aiming to interfere with specific targets important in the break of tolerance and/or persistence of the autoimmune response might further improve the prognosis of autoimmune vasculitides such as antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV). In vitro and in vivo studies suggest that the interaction of ANCA and cytokine (TNF-α, IL-1)-primed neutrophils results in premature neutrophil activation and degranulation, subsequent endothelial cell damage, and further leukocyte recruitment. For one of the AAV, Wegener's granulomatosis, recent ex vivo data have provided evidence that WG-granulomata might provide the necessary "proinflammatory environment" for the break of tolerance and display features of lymphoid-like tissue neoformation, in which autoimmunity to "Wegener's autoantigen" proteinase 3 PR3 could be sustained. Blocking TNF-α and eliminating autoreactive B cells seem promising treatment targets to interfere with these fundamental disease processes. While the recombinant TNF-α receptor/IgG1 fusion protein etanercept, in addition to standard therapy with subsequent tapering of standard medications, was found to be not effective for maintenance of remission, open clinical studies suggest a beneficial effect of the anti-TNF-α antibody infliximab in addition to standard therapy for the induction of remission in patients with refractory AAV. Peripheral B cell depletion with the anti-CD20 antibody rituximab also induced remissions in AAV in uncontrolled trials.

Original languageEnglish
Title of host publicationAutoimmunity, Part B Novel Applications of Basic Research
Number of pages10
PublisherBlackwell Publishing Inc.
Publication date09.2007
ISBN (Print)1573317098, 9781573317092
Publication statusPublished - 09.2007

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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