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Extended report
Damage in the anca-associated vasculitides: long-term data from the European Vasculitis Study group (EUVAS) therapeutic trials
  1. Joanna Robson1,2,
  2. Helen Doll3,
  3. Ravi Suppiah4,
  4. Oliver Flossmann5,
  5. Lorraine Harper6,
  6. Peter Höglund7,
  7. David Jayne8,
  8. Alfred Mahr9,
  9. Kerstin Westman10,
  10. Raashid Luqmani1
  1. 1Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, USA
  2. 2Rheumatology Department, Nuffield Orthopaedic Centre, Oxford, UK
  3. 3Department of Population Health, Norwich Medical School, Faculty of Medicine and Health Sciences, University of East Anglia, Norwich, UK
  4. 4Department of Rheumatology, Auckland District Health Board, Auckland, New Zealand
  5. 5Renal Department, Royal Berkshire NHS Foundation Trust, Reading, UK
  6. 6School of Immunity and Infection, Centre for Translational Inflammation Research, University of Birmingham, Birmingham, UK
  7. 7Competence Centre for Clinical Research, Skane University Hospital, Lund, Sweden
  8. 8Renal Department, Addenbrooke's Hospital, Cambridge, UK
  9. 9Department of Internal Medicine, Hospital Saint-Louis, Paris, France
  10. 10Nephrology and Transplantation, Skane University Hospital Malmo, Lund University, Malmo, Sweden
  1. Correspondence to Dr Joanna Robson, Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford; Rheumatology Department, Nuffield Orthopaedic Centre, Windmill Road, Oxford, OX3 7LD, UK. joanna.robson{at}


Objectives To describe short-term (up to 12 months) and long-term (up to 7 years) damage in patients with newly diagnosed antineutrophil-cytoplasm antibody-associated vasculitis (AAV).

Methods Data were combined from six European Vasculitis Study group trials (n=735). Long-term follow-up (LTFU) data available for patients from four trials (n=535). Damage accrued was quantified by the Vasculitis Damage Index (VDI). Sixteen damage items were defined a priori as being potentially treatment-related.

Results VDI data were available for 629 of 735 patients (85.6%) at baseline, at which time 217/629 (34.5%) had ≥1 item of damage and 32 (5.1%) ≥5 items, reflecting disease manifestations prior to diagnosis and trial enrolment. LTFU data were available for 467/535 (87.3%) at a mean of 7.3 years postdiagnosis. 302/535 patients (56.4%) had VDI data at LTFU, with 104/302 (34.4%) having ≥5 items and only 24 (7.9%) no items of damage. At 6 months and LTFU, the most frequent items were proteinuria, impaired glomerular filtration rate, hypertension, nasal crusting, hearing loss and peripheral neuropathy. The frequency of damage, including potentially treatment-related damage, rose over time (p<0.01). At LTFU, the most commonly reported items of treatment-related damage were hypertension (41.5%; 95% CI 35.6 to 47.4%), osteoporosis (14.1%; 9.9 to 18.2%), malignancy (12.6%; 8.6 to 16.6%), and diabetes (10.4%; 6.7 to 14.0%).

Conclusions In AAV, renal, otolaryngological and treatment-related (cardiovascular, disease, diabetes, osteoporosis and malignancy) damage increases over time, with around one-third of patients having ≥5 items of damage at a mean of 7 years postdiagnosis.

  • Disease Activity
  • Epidemiology
  • Granulomatosis with polyangiitis
  • Outcomes research
  • Treatment

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