L Engineer1, A R Ahmed. 1. Department of Oral Medicine and Diagnostic Sciences, Harvard School of Dental Medicine, Boston, MA 02115, USA.
Abstract
BACKGROUND: Conventional therapy of severe bullous pemphigoid (BP) relies on the use of high-dose systemic corticosteroids with or without adjuvants, notably immunosuppressive agents. This approach can result in debilitating and potentially fatal side effects, hence the need to explore alternative therapies. Intravenous immunoglobulin (IVIg) therapy is emerging as one possibility. OBJECTIVE: Our purpose was to determine whether any preliminary conclusion can be drawn about the potential for the use of IVIg to treat patients with severe BP. METHODS: A literature search was done to identify reports, in English-language peer-reviewed journals, on the use of IVIg to treat patients with BP. Reports were examined for information on disease duration, severity, therapies used before and after IVIg, dose and frequency of IVIg administration, and its immediate and long-term effects. RESULTS: Data on treatment of 17 patients indicated that 12 patients (70%) experienced a beneficial clinical response to IVIg. In 5 patients (30%) no clinical benefit was observed. A minimum dose of 2 g/kg per cycle at monthly intervals for 3 months has been the most common approach. However, this should not be perceived as a "standard dose" at the present time. In some patients the use of IVIg appears to permit a systemic corticosteroid-sparing effect. Longer use has achieved sustained clinical remission in some patients. Lack of response was observed in patients who received low-dose IVIg or who received a single infusion only. Minimal side effects in the form of headaches and nausea and vomiting were observed in some patients. CONCLUSION: The present experience consists only of open uncontrolled trials in a few patients and does not allow for definitive conclusions. However, at this time IVIg appears to be a promising agent for the treatment of BP, especially for patients who do not respond to conventional therapy. Adequate doses for longer periods may be required to induce and maintain sustained clinical remissions. Large-scale controlled studies with defined entry criteria, objectives, end points, and long-term follow-up are necessary to determine the specific role of IVIg in the overall management of BP.
BACKGROUND: Conventional therapy of severe bullous pemphigoid (BP) relies on the use of high-dose systemic corticosteroids with or without adjuvants, notably immunosuppressive agents. This approach can result in debilitating and potentially fatal side effects, hence the need to explore alternative therapies. Intravenous immunoglobulin (IVIg) therapy is emerging as one possibility. OBJECTIVE: Our purpose was to determine whether any preliminary conclusion can be drawn about the potential for the use of IVIg to treat patients with severe BP. METHODS: A literature search was done to identify reports, in English-language peer-reviewed journals, on the use of IVIg to treat patients with BP. Reports were examined for information on disease duration, severity, therapies used before and after IVIg, dose and frequency of IVIg administration, and its immediate and long-term effects. RESULTS: Data on treatment of 17 patients indicated that 12 patients (70%) experienced a beneficial clinical response to IVIg. In 5 patients (30%) no clinical benefit was observed. A minimum dose of 2 g/kg per cycle at monthly intervals for 3 months has been the most common approach. However, this should not be perceived as a "standard dose" at the present time. In some patients the use of IVIg appears to permit a systemic corticosteroid-sparing effect. Longer use has achieved sustained clinical remission in some patients. Lack of response was observed in patients who received low-dose IVIg or who received a single infusion only. Minimal side effects in the form of headaches and nausea and vomiting were observed in some patients. CONCLUSION: The present experience consists only of open uncontrolled trials in a few patients and does not allow for definitive conclusions. However, at this time IVIg appears to be a promising agent for the treatment of BP, especially for patients who do not respond to conventional therapy. Adequate doses for longer periods may be required to induce and maintain sustained clinical remissions. Large-scale controlled studies with defined entry criteria, objectives, end points, and long-term follow-up are necessary to determine the specific role of IVIg in the overall management of BP.