Rheumatology Advance Access originally published online on May 31, 2008
Rheumatology 2008 47(8):1111-1113; doi:10.1093/rheumatology/ken202
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
EDITORIALS |
Germinal centre-like structures in Wegener's granuloma: the morphological basis for autoimmunity?
1Department of Rheumatology and 2Institute of Pathology, University of Luebeck, Luebeck, Germany
Correspondence to: A. Mueller, Department of Rheumatology, University of Luebeck, Ratzeburger Allee 160, D-23538 Luebeck, Germany. E-mail: mueller@mail.uni-luebeck.de
| The first 150 words of the full text of this article appear below. |
Wegener's granulomatosis (WG) shows a unique dual morphology in comprising a vasculitis as well as extravascular granulomatous inflammation. Together with the much rarer Churg–Strauss syndrome, WG is the only primary systemic vasculitis that features a salient extravascular inflammatory component. While the pathophysiology of ANCA-induced endothelial vessel wall damage has been well studied (for review see [1]), more recent data described the formation of lymphatic follicles in granulomatous lesions and their potential role for the generation of ANCA against Wegener's autoantigen proteinase 3 (PR3-ANCA) [2, 3]. Further, an intricate interplay between cells and molecules of the innate and adaptive immune response resulting in chronic inflammation has been elucidated for WG, lately [4–7]. From these and other findings, a pathogenetic and pathophysiological scenario emerges, in which multiple steps of the immune pathology leading from granulomatous, autoantibody-negative to vasculitic, autoantibody-positive WG are being dissected [8
Morphological spectrum of Wegener's granuloma
Germinal centre-like structures in Wegener's granuloma: inducing and sustaining autoimmunity?
Exogenous and endogenous factors governing granuloma formation and autoimmunity to PR3 in WG