The patient outcome from ANCA-associated vasculitides, Wegener’s granulomatosis (WG), Microscopic Polyangiitis (MPA) and Renal Limited Vasculitis (RLV), improved dramatically after the introduction of cyclophosphamide and corticosteroids in the 1970’ies. However, the dosages of the immunosuppressive therapy have been revised, diagnosis has been facilitated by serology and the care of patients has improved during the last decades. There are several studies presenting data on outcome, however using varying diagnostic criteria, diverging in phenotypes, differing in therapy and comprising a limited number of patients. Data on outcome are needed when designing new therapeutic trials, and thus updated data from the clinical trials NORAM, CYCAZAREM, CYCLOPS and MEPEX has been assessed.

The primary aim is to estimate patient and renal survival, the frequency of disease relapse and the accumulation of disease related damage, at a five-year follow-up in the cohort of patients, with ANCA-associated vasculitis, who have participated in any of the first four EUVAS-trials.

The second aim is to assess the co-morbidity with diabetes mellitus, cardiovascular events, malignancies and other severe adverse events in the same cohort of patients.

The third aim is to identify possible prognostic factors at entry, remission and 12 months, respectively on the long-term outcomes.

A questionnaire was sent out to the participating centres. Data on renal function, cumulative incidence of cardiovascular events, malignancies, fractures and patient survival were obtained, and duration and type of immunosuppression after the closure of the respective therapeutic clinical trial.

The first long-term follow-up study defining the survival of patients with ANCA-associated vasculitis has been accepted for publication in 2011.



Kerstin Westman


1. Long-term mortality

2. Relapses

3. Cardiovascular morbidity

4. Cumulative incidence on malignancy

5. Adverse events

6. Renal outcome and histology

7. Evaluation of scoring instruments

8. Evaluation of quality of life index (SF-36)

9. Surrogate markers for outcome

10. ENT damage in Wegener’s granulomatosis