L Sagi1, S Baum, V Gendelman, H Trau, A Barzilai. 1. Department of Dermatology, Sheba Medical Center, Affiliated to Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. lior115@gmail.com
Abstract
BACKGROUND: The treatment of pemphigus, an autoimmune bullous disease, is based on the combination of corticosteroids and adjuvant therapies, such as immunosuppressive drugs, anti-inflammatory drugs and immunomodulatory procedures, such as intravenous immunoglobulin and therapeutic plasma exchange (TPE). OBJECTIVE: This study aims to assess our experience with TPE as a steroid-sparing modality in moderate and severe intractable pemphigus patients. METHODS: A retrospective evaluation for all intractable pemphigus patients treated by TPE in a university-affiliated tertiary referral medical centre between the years 1998 and 2008. Treatment protocol included three TPE treatments weekly for 1-3 months, combined with monthly pulse therapy of dexamethasone and/or cyclophosphamide. Maintenance therapy was based on once/bi weekly TPE treatments or monthly intravenous immunoglobulin. RESULTS: Seven patients were included in the study, four with severe pemphigus vulgaris and three with moderate disease. Six of the seven patients responded to TPE: Four patients (57%) achieved complete remission and two patients (28%) achieved partial remission on minimal therapy. Mild adverse effects related to TPE were observed in two patients and included dizziness and mild headache. CONCLUSION: TPE is a well-tolerated effective steroid-sparing agent in recalcitrant pemphigus patients.
BACKGROUND: The treatment of pemphigus, an autoimmune bullous disease, is based on the combination of corticosteroids and adjuvant therapies, such as immunosuppressive drugs, anti-inflammatory drugs and immunomodulatory procedures, such as intravenous immunoglobulin and therapeutic plasma exchange (TPE). OBJECTIVE: This study aims to assess our experience with TPE as a steroid-sparing modality in moderate and severe intractable pemphigus patients. METHODS: A retrospective evaluation for all intractable pemphigus patients treated by TPE in a university-affiliated tertiary referral medical centre between the years 1998 and 2008. Treatment protocol included three TPE treatments weekly for 1-3 months, combined with monthly pulse therapy of dexamethasone and/or cyclophosphamide. Maintenance therapy was based on once/bi weekly TPE treatments or monthly intravenous immunoglobulin. RESULTS: Seven patients were included in the study, four with severe pemphigus vulgaris and three with moderate disease. Six of the seven patients responded to TPE: Four patients (57%) achieved complete remission and two patients (28%) achieved partial remission on minimal therapy. Mild adverse effects related to TPE were observed in two patients and included dizziness and mild headache. CONCLUSION: TPE is a well-tolerated effective steroid-sparing agent in recalcitrant pemphigus patients.