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There is continuous debate about whether Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg–Strauss syndrome (CSS) represent one disease entity—‘antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV)’ (eg, as presented by Hogan et al1) or whether they constitute separate diseases which share common features but are otherwise heterogeneous—a view held, for example, by Hoffman and Langford.2 ANCA are a common finding in AAV, yet the majority of patients with CSS are ANCA negative. Several clinical and histopathological features unique to one disease (eg, asthma for CSS) support Hoffman's opinion. Analysis of the genetic background of the three disorders may help to differentiate between both concepts. The strongest genetic association for WG pertains to the HLA-DPB1 region,3 ,4 an association not seen in CSS.5 For MPA HLA-DPB1 …
LA, JUH contributed equally to this work.
Funding This work was supported by the Deutsche Forschungsgemeinschaft (KFO 170).
Competing interests None.
Ethics approval This study was conducted with the approval of the Universities of Lübeck, Germany and Birmingham, UK.
Provenance and peer review Not commissioned; externally peer reviewed.
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