Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment

Background: Up to 24% of patients with bullous pemphigoid (BP) do not respond to conventional therapy consisting of oral prednisone alone or combined with corticosteroid-sparing immunosuppressive agents (ISAs). They cannot sustain a prolonged clinical remission and continue to have relapses. Objective: Fifteen patients with recurrent BP who had experienced several significant side effects resulting from conventional therapy were treated with intravenous immunoglobulin (Mg) therapy. Methods: A preliminary dose-finding study tested 7 additional patients to ascertain the optimal IVIg dose of 2 gm/kg per cycle. Objective parameters to determine clinical outcomes were recorded before and after IVIg therapy: doses of prednisone and ISAs, their duration, side effects, clinical course, frequency of relapses and recurrence, response to therapy, number of hospitalizations, total days hospitalized, and quality of life. Results. While receiving Mg as monotherapy all study subjects achieved a sustained clinical remission. A statistically significant difference was noted in all the variables studied before and after Mg therapy. IVIg had a corticosteroid-sparing effect and improved quality of life and did not produce any serious side effects. Conclusion: IVIg appears to be an effective alternative in treating patients with severe BP whose disease is nonresponsive to conventional therapy. IVIg may be particularly useful, if treatment is begun early, in patients who are at risk of experiencing serious or potentially fatal side effects from conventional immunosuppressive therapy After clinical control is achieved, IVIg therapy should be gradually withdrawn and not abruptly discontinued.