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.