This medical condition has either been superseded or has become inactive
| 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 |
Pemphigus vulgaris (PV) is a rare but potentially fatal condition accounting for approximately 70% of pemphigus cases. While the cause is unknown, an immuno-genetic predisposition is well established. PV may also be drug-induced. Drugs reported to be most significantly associated with PV include penicillamine, captopril and other thiol-containing compounds. Rifampicin and emotional stress have recently been reported as triggers for PV. The oral cavity is almost always affected and erosions can be scattered and extensive, with subsequent dysphagia. Blistering and erosions secondary to the rupture of blisters may be painful and limit the patient’s daily activities. Pemphigus may occur in patients with other autoimmune diseases, particularly myasthenia gravis and thymoma. Prognosis The severity and natural history of PV are variable. Before the advent of steroids, most patients with PV died. Treatment with systemic steroids has reduced the mortality rate to 5–15%. Most deaths occur during the first few years of disease and, if the patient survives five years, the prognosis is good. Early disease is easier to control than widespread disease, and mortality may be higher if therapy is delayed. Morbidity and mortality are related to the extent of disease, the maximum dose of corticosteroid required to induce remission, and the presence of other diseases. |
| Justification for Evidence Category |
In a retrospective cohort study, 15 corticosteroid-dependent patients with moderate to severe PV were treated with intravenous immunoglobulin (IVIg) and followed over a mean period of 6.2 years. All 15 patients had a satisfactory clinical response to IVIg therapy. IVIg had a demonstrable corticosteroid-sparing effect and was considered a safe and effective alternative treatment in patients who were dependent on systemic corticosteroids or who developed significant adverse effects as a result of their use (Biotext 2004). |
| Diagnosis Requirements |
A diagnosis must be made by a Dermatologist. |
| Qualifying Criteria for Ig Therapy |
AND
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| Review Criteria for Assessing the Effectiveness of Ig Use |
Review is required every six months by a Dermatologist and improvement must be demonstrated for continuation of supply.
On review of the initial authorisation period Evidence of clinical effectiveness of Ig therapy, and criteria for continued use may include:
On review of a continuing authorisation period Evidence of clinical effectiveness of Ig therapy, and criteria for continued use may inlcude:
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| Dose |
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. Refer to the current product information sheet for further information. |
| Bibliography |
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| Biotext 2004, ‘Summary data on conditions and papers’, 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. 240–1. Available from: http://www.nba.gov.au/pubs/pdf/report-lit-rev.pdf Bystryn, JC, Jiao, D & Natow, S 2002, ‘Treatment of pemphigus with intravenous immunoglobulin’, Journal of the American Academy of Dermatology, vol. 47, no. 3, pp. 358–63. Sami, N, Oureshi, A, Ruocco, E, et al 2002, ‘Corticosteroid-sparing effect of intravenous immunoglobulin therapy in patients with pemphigus vulgaris’, Archives of Dermatology, vol. 138, pp. 1158–62. |