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Journal of the European Academy of Dermatology and Venereology : JEADV · 2011

The role of therapeutic plasma exchange in pemphigus vulgaris.

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Sagi L, Baum S, Gendelman V, Trau H, Barzilai A

Аннотация

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.

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