Qualifying Criteria Moderate to severe disease diagnosed by a dermatologist; AND Corticosteroids or immunosuppressive agents are contraindicated; OR Condition is unresponsive to corticosteroids and immunosuppressive agents; OR Presenting with severe side effects of therapy. Moderate to severe CP/MMP disease proven by biopsy and serology with involvement of multiple sites, oesophageal involvement alone or conjunctiva alone. AND Persistent disease despite standard corticosteroid and treatment with at least 2 immunosuppressant agents or B cell depletion therapy (Rituximab) OR Severe side effects prohibit the continuation of corticosteroids and immunosuppressive agents unless corticosteroids or immunosuppressant agents are contraindicated. Qualifying criteria have been expanded to require diagnostic confirmation and appropriate disease required for ‘moderate to severe disease’. (A) Criteria are consistent with original version with details defined. (A) E.g. immunosuppressant therapeutics that need to have been tried include: Corticosteroids Azathioprine Cyclophosphamide Methotrexate Mycophenolate Rituximab Severe side effects of immunosuppressant therapy include: Significant infection including sepsis Malignancy Marrow suppression and cytopenia Unstable Diabetes Severe osteoporosis History of avascular necrosis Review Criteria Response demonstrated at review at six months. Improvement to be demonstrated for continuation of supply. Disease recurrence or relapse and duration of clinical remission. Review is required every six months by a dermatologist or clinical immunologist. Response must be demonstrated at the initial review at six months and improvement must be demonstrated for continuation of supply. Consideration should be given to a trial-off immunoglobulin (Ig) therapy once the patient has achieved stabilised disease or clinical Initial review to confirm response is unchanged, however, ongoing six monthly review has been added with specific criteria defined for ongoing treatment. (A) Consideration is now to trial off therapy once disease is stable and/or reduce Ig dose.
Dosing above 1 g/kg per day is contraindicated for some IVIg products. to the current product information sheet for further information. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Consideration should be given to a trial-off immunoglobulin (Ig) therapy once the patient has achieved stabilised disease or clinical remission. The minimal effective dose should be prescribed. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Dosing above 1 g/kg per day is contraindicated for some IVIg products. to the current product information sheet for further information. indicate two weekly dosing is supported when eyesight is threatened. (A) BIBLIOGRAPHY AR & Dahl, MV, for the Consensus Development Group 2003, ‘Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mucocutaneous blistering diseases’, Archives of Dermatology, vol. 139, pp. 1051–9. Daoud, YJ & Amin, KG 2006, ‘Comparison of cost of immune globulin intravenous therapy to conventional immunosuppressive therapy in treating patients with autoimmune mucocutaneous blistering diseases’, International Immunopharmacology, vol. 6, no. 4, pp. 600–6. Letko, E, Miserocchi, E, Daoud, YJ, et al 2004, ‚A nonrandomized comparison of the clinical outcome of ocular involvement in patients with mucous membrane (cicatricial) pemphigoid between conventional immunosuppressive and intravenous immunoglobulin therapies’, Clinical Immunology, vol. 111, no. 3, pp. 303–10. Orange, JS, et al 2006, 'Use of intravenous immunoglobulin in human disease: A review of primary evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology’, Journal of Allergy and Clinical Immunology, vol. 117, no. 4, pp. S525–53. END OF DOCUMENT i