Specific Conditions |
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Indication for Ig Use |
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Level of Evidence | Evidence of probable benefit – more research needed (Category 2a) |
Description and Diagnostic Criteria |
Anti-neutrophil cytoplasmic antibody (ANCA) associated systemic necrotising vasculitides are life-threatening immune-mediated inflammatory diseases comprising one of four clinical syndromes:
Standard combinations of corticosteroids and cytotoxic immunosuppression are generally effective at controlling disease, but relapses are common. |
Justification for Evidence Category |
The Biotext (2004) review found one randomised trial of 34 patients and one case series of seven patients with ANCA-associated systemic vasculitis (AASV). Different AASVs were represented in the studies. The Biotext (2004) review concluded that there is possible benefit in the treatment of AASV with IVIg if disease activity persists after standard therapy. However, in recent years, there have been a number of randomised controlled trials demonstrating the effectiveness of immunosuppressants and biologicals in achieving remission and treating relapsing and refractory disease. In particular, Rituximab is now considered a mainstay of treatment, and is now available on the Australian Pharmaceutical Benefits Schedule (PBS). The publication of the evidence based British Guidelines in 2014 for treatment of ANCA in adults, do not mention Ig therapy. It is recognized that Rituximab is more effective than Cyclosporine in refractory disease and if the patient has not had previous treatment with Rituximab, then it is the first choice. Ig therapy is only indicated in patients who have failed to respond to increased doses of immunosuppressants and a further trial of Rituximab, or in those in whom these therapies are contraindicated. |
Diagnosis Requirements |
A diagnosis must be made by an Immunologist, Nephrologist or a Rheumatologist. |
Qualifying Criteria for Ig Therapy |
Anti-neutrophil cytoplasmic antibody (ANCA) positive systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression
This indication should be used for new patients and those that have never received Ig therapy for this indication. For responding patients who have relapsed after previous Ig therapy please use the indication Relapse in Anti-neutrophil cytoplasmic antibody (ANCA) positive systemic necrotising vasculitis resistant following response to Ig therapy.
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Relapse in ANCA positive systemic necrotising vasculitis resistant following response to Ig therapy
This indication should be used for responding anti-neutrophil cytoplasmic antibody (ANCA) positive systemic necrotising vasculitis patients who have relapsed following previous treatment with Ig therapy. For new patients and those that have never received Ig therapy, please use the indication ANCA positive systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression.
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Exclusion Criteria |
First-line or initial treatment for ANCA |
Review Criteria for Assessing the Effectiveness of Ig Use |
Anti-neutrophil cytoplasmic antibody (ANCA) positive systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression
Six months treatment is authorised for patients in the first instance. While review is not mandated for this condition, reporting of clinical outcome data is strongly encouraged as demonstrated clinical response to Ig therapy is required for eligibility for further authorisation, should the patient relapse in the future.
Clinical effectiveness of Ig therapy may be assessed by:
Relapse in ANCA positive systemic necrotising vasculitis resistant following response to Ig therapy
Six monthly review by a rheumatologist, immunologist or nephrologist is required to assess evidence of clinical benefit. Once the patient has been in clinical remission for two years after relapse, cessation of Ig therapy should be considered.
Clinical effectiveness of Ig therapy may be assessed by: On review of the initial authorisation period
On review of a continuing authorisation period
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Dose |
Anti-neutrophil cytoplasmic antibody (ANCA) positive systemic necrotising vasculitis failing to respond to corticosteroids and cytotoxic immunosuppression
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindications. Relapse in ANCA positive systemic necrotising vasculitis resistant following response to Ig therapy
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindications. |
Bibliography |
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Biotext 2004, ‘Summary data on conditions and papers’, in A systematic literature review and report on the efficacy of intravenous immunoglobulin therapy and its risks, commissioned by the National Blood Authority on behalf of all Australian Governments, pp. 248–50. Available from: https://www.blood.gov.au/system/files/A-systematic-literature-review-and-report-on-the-efficacy-of-IVIg-therapy-and-its-risks.pdf Foster, R, Rosenthal, E, Marques, S, et al 2006, ‘Primary systemic vasculitis: treatment of difficult cases’, Lupus, vol. 15, no. 3, pp. 143–7. https://www.ncbi.nlm.nih.gov/pubmed/16634367 Jayne, DR, Davies, MJ, Fox, CJ, et al 1991, ‘Treatment of systemic vasculitis with pooled intravenous immunoglobulin’, Lancet, vol. 337, no. 8750, pp. 1137–9 Jayne, DR & Rasmussen, N 1997, ‘Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community Systemic Vasculitis Clinical Trials Study Group’, Mayo Clinic Proceedings, vol. 72, no. 8, pp. 737–47. http://www.mayoclinicproceedings.org/article/S0025-6196(11)63594-5/abstract Jayne, DR, Chapel, H, Adu, D, et al 2000, ‘Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity’, Quarterly Journal of Medicine, vol. 93, no. 7, pp. 433–9. https://www.ncbi.nlm.nih.gov/pubmed/10874052 |