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” |