Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials.
OBJECTIVE
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.
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, USA Rheumatology Department, Nuffield Orthopaedic Centre, Oxford, UK.
Joanna
J
Robson
Department of Population Health, Norwich Medical School, Faculty of Medicine and Health Sciences, University of East Anglia, Norwich, UK.
Helen
H
Doll
Department of Rheumatology, Auckland District Health Board, Auckland, New Zealand.
Ravi
R
Suppiah
Renal Department, Royal Berkshire NHS Foundation Trust, Reading, UK.
Oliver
O
Flossmann
School of Immunity and Infection, Centre for Translational Inflammation Research, University of Birmingham, Birmingham, UK.
Lorraine
L
Harper
Competence Centre for Clinical Research, Skane University Hospital, Lund, Sweden.
Peter
P
Höglund
Renal Department, Addenbrooke's Hospital, Cambridge, UK.
David
D
Jayne
Department of Internal Medicine, Hospital Saint-Louis, Paris, France.
Alfred
A
Mahr
Nephrology and Transplantation, Skane University Hospital Malmo, Lund University, Malmo, Sweden.
Kerstin
K
Westman
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, USA.
Raashid
R
Luqmani
D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000188:drug therapy; D003920:Diabetes Mellitus / Q000209:etiology; D018450:Disease Progression; D005060:Europe; D005260:Female; D005919:Glomerular Filtration Rate; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D006973:Hypertension / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000009:adverse effects; D008297:Male; D055953:Microscopic Polyangiitis / Q000150:complications* / Q000188:drug therapy; D008875:Middle Aged; D015508:Nasal Obstruction / Q000209:etiology*; D009369:Neoplasms / Q000209:etiology; D010024:Osteoporosis / Q000209:etiology; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D011507:Proteinuria / Q000209:etiology*; D012720:Severity of Illness Index
D007166:Immunosuppressive Agents
Disease Activity; Epidemiology; Granulomatosis with polyangiitis; Outcomes research; Treatment