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  1. Annelies Berden, resident in internal medicine1,
  2. Arda Göçeroğlu, research fellow1,
  3. David Jayne, consultant in nephrology and vasculitis 2,
  4. Raashid Luqmani, professor of rheumatology 3,
  5. Niels Rasmussen, senior consultant in otolaryngology4,
  6. Jan Anthonie Bruijn, professor of immunopathology1,
  7. Ingeborg Bajema, nephropathologist 1
  1. 1Department of Pathology, Leiden University Medical Centre, Leiden, 2300 RC, Netherlands
  2. 2Renal Unit, Addenbrooke’s Hospital, Cambridge, UK
  3. 3Department of Rheumatology, University of Oxford, Oxford, UK
  4. 4Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark
  1. Correspondence to: I Bajema i.bajema{at}lumc.nl

Summary points

  • Consider antineutrophil cytoplasmic antibody (ANCA) associated vasculitis when inflammatory disease cannot be ascribed to any other disease and inflammation progresses despite antibiotics

  • Avoid diagnostic delay to prevent end organ damage, particularly renal disease

  • Test for ANCA in patients with chronic destructive upper airway disease, pulmonary nodules, renal and pulmonary inflammatory disease, rapidly progressive glomerulonephritis, skin vasculitis with systemic illness, mononeuritis multiplex, subglottic stenosis of the trachea, and retro-orbital mass

  • Patients should be managed by a specialist in vasculitides

  • Remission is usually induced with high dose glucocorticoids and cyclophosphamide, followed by remission maintenance treatment

  • Adverse responses to treatment are common, as are relapses, so long term follow-up is needed

Vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) are systemic autoimmune diseases of unknown cause that affect small to medium sized blood vessels. They include granulomatosis with polyangiitis (formerly Wegener’s granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). This review mainly focuses on granulomatosis with polyangiitis and microscopic polyangiitis. Although they are relatively rare, they must be diagnosed and treated early because untreated disease may rapidly develop into multiple organ failure and death. With modern treatment, these diseases are no longer fatal but chronic. Early diagnosis and treatment may prevent progression to end organ damage and lengthen healthier life. A recent large survey of patients with ANCA associated vasculitis found a lag of three to 12 months between disease onset and diagnosis, suggesting that diagnostic delay is a problem.1 We review the diagnosis and management of ANCA associated vasculitides for the generalist reader, drawing on the findings of observational studies, randomised controlled trials, and meta-analyses.

Sources and selection criteria

We searched PubMed (original search performed in August 2011, updated in December 2011) for relevant articles on epidemiology, diagnosis, and management of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis. Where possible, we sought data from …

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