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Are there specific genetic risk factors for the different forms of ANCA-associated vasculitis?
  1. Larissa Arning1,
  2. Julia Ulrike Holle2,3,
  3. Lorraine Harper4,
  4. David S Millar5,
  5. Wolfgang Ludwig Gross2,3,
  6. Jörg Thomas Epplen1,
  7. Stefan Wieczorek1
  1. 1Ruhr-University, Human Genetics, Bochum, Germany
  2. 2Department of Rheumatology, University Hospital Schleswig-Holstein, Campus Luebeck, Luebeck, Germany
  3. 3Department of Rheumatology and Immunology, Klinikum Bad Bramstedt, Bad Bramstedt, Germany
  4. 4Division of Immunity and Infection, University of Birmingham, Edgbaston, Birmingham, UK
  5. 5Institute of Medical Genetics, Cardiff University, Cardiff, UK
  1. Correspondence to Dr Stefan Wieczorek, Ruhr University, Human Genetics, MA5/39 44780 Bochum, Germany; stefan.wieczorek{at}rub.de

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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 association …

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