Pemphigus : A Treatment Update

摘要: Pemphigus is an uncommon but potentially life-threatening chronic autoimmune bullous disorder. Antibodies are directed against antigens (desmoglein 1 and 3) in the desmosomes linking keratinocytes acetylcholine receptors. Conventional treatment with high-dose corticosteroids, sometimes adjuvant immunosuppressive agents, may be associated very serious adverse effects. There urgent need to establish evidence for safest most effective form of treatment. A literature review has revealed 11 controlled (9 randomized) trials pemphigus. The numbers participants individual small data cannot pooled as they evaluate different forms results these suggest that high doses either pulse therapy or daily dosage, not superior moderate doses. Based on from available trials, addition agent generally does appear offer substantial benefit terms clinical response. However, a recent study demonstrated significant reduction corticosteroid requirements among patients receiving agents. Newer therapies, such biologic agents (in particular rituximab), calcineurin inhibitors, immunoadsorption promising there inadequate their role clearly. Initial open-label studies specific peptide immunotherapy safe novel approach pemphigus future. At present, patient requires judgment should based purely guidelines alone. large randomized, controlled, multicenter