Table 1.: Main results from the needs assessment questionnaire on practice patterns for management of ANCA-associated vasculitis (number of respondents seeing at least 1 vasculitis patient = 132).

Questionnaire Items Answers* Comments
  Always Never  
Diagnostic investigations
Which laboratory investigations for patients with suspected AAV?    
Complete blood count 100% 0
Liver function test 94% 0
Renal function test 100% 0
C-reactive protein 88% 1%
ESR 87% 0
Urinalysis (routine and microscopic analysis) 98% 1%
Von Willebrand factor antigen 7% 58%
Complement C3/C4 76% 2%
ANCA 91% 6% “Some refuse ANCA testing because of the cost”
Antinuclear autoantibody 85% 0
Serum protein electrophoresis 47% 11%
Lupus anticoagulant and/or anticardiolipin 42% 6%
Which other investigations for patients with suspected AAV? “Depends on clinical presentation”
Chest X-ray 96% 0
Tuberculin skin test 25% 11%
Tuberculosis interferon assay (if available) 3% 47%
Pulmonary function tests 45% 2%
Bronchoscopy + bronchoalveolar lavage 16% 7%
Biopsy of involved organ system 48% 1%
CT scan /MRI of involved organ 57% 0
Echocardiogram 30% 2%
Cardiac MRI (EGPA) 7% 31%
IgE level (EGPA) 52% 4%
Electromyogram 4% 11%
Induction treatments
Which of the following treatments do you use to induce remission?
Corticosteroids (intravenous pulse) 94% 6%
Oral corticosteroids 94% 0%
Oral cyclophosphamide 87% 13%
Intravenous cyclophosphamide 91% 9% “IV cyclophosphamide not infused at my centre”,
“initial IV cyclophosphamide pulse then switch to daily oral”
Methotrexate 64% 36% “methotrexate and azathioprine for mild/limited disease”
Azathioprine 47% 53%
Mycophenolate mofetil 33% 67%
Rituximab 68% 32% “would use rituximab if approved”†
Leflunomide 10% 90%
Anti–tumor necrosis factor alpha agent(s) 7% 93%
Plasma Exchange 48% 52%
Maintenance treatment
What medications do you use?
Methotrexate 86% 14%
Azathioprine 96% 4%
Cyclophosphamide (oral) 23% 77%
Rituximab 32% 68% “would use rituximab if could get it before relapse”
Infliximab 3% 97%
Leflunomide 17% 83%
Mycophenolate mofetil 53% 47%
Trimethoprim / sulfamethoxazole (160/800 mg) twice daily 60% 40%
Follow-up
Which of the following tests to monitor your patients?
Complete blood count 100% 0
Liver function tests 86% 2%
Creatinine 98% 0
ANCA 57% 7%
ESR 79% 1%
C- reactive protein 84% 3%
Urinalysis 91% 1%
CT scan of chest and/or sinus 17% 6%
Pulmonary function tests 30% 7%
Electromyogram 0 41%
Bone mineral density 49% 6%
CD19+ B cell count (in patients who received rituximab) 14% 67%
Urine cytology (patients who received cyclophosphamide) 43% 22%
Cystoscopy (patients who received cyclophosphamide) 9% 33% “cystoscopy only if persistent microscopic hematuria”

Data are percentage of respondents.
* Respondents could skip questions, or chose between Always / Sometimes / Never.
At the time the survey was conducted, rituximab was not yet approved in all Canadian provinces for induction in adult patients with severe AAV (and certain other specific criteria that may vary according to each province).
AAV: ANCA-associated vasculitis; ANCA: anti-neutrophil cytoplasmic antibodies; EGPA: eosinophilic granulomatosis with polyangiitis; ESR: erythrocyte sedimentation rate.