Plasmapheresis therapy in pemphigus vulgaris and bullous pemphigoid

Follicular Non-Hodgkin's Lymphoma with Refractory Paraneoplastic Pemphigus: Case Report with Review of Novel Treatment Modalities

“…Plasmapheresis or plasma exchange in combination with corticosteroids and/or cyclophosphamide or azathioprine for prevention of rebound flare of disease showed similar rapid and beneficial results in association with decreasing auto-antibody levels in this group of refractory pemphigus, leading to reduced dosage of these drugs in time [11,13,14,23,24]. Synchronization of PE with subsequently IVIg therapy is theoretically even more powerful and has been used successfully in patients refractory to PE and immunosuppressants [14].…”

“…The patients sometimes benefit from high doses of oral corticosteroids [15]. However, pulse therapy with high doses of prednisolone (or DXM) in combination with other immunosuppressant agents like azathioprine, mycophenolate mofetyl or cyclophosphamide induces variable and inconstant results [6,10,11].…”

Drug‐resistant bullous pemphigoid and inflammatory bowel disease in a pediatric case successfully treated by plasma exchange and extracorporeal photochemotherapy

“…Despite much research and the development of many new therapies, successful treatment strategies for patients who are resistant to first-line corticosteroid therapy or to reduced corticosteroid approaches have not been identified. Two studies [13,14] showed the benefit of plasmapheresis as steroid saving agent; although the literature regarding the use of plasmapheresis in drug-resistant BP is scant [2,3,[15][16][17], it does show that a general improvement in clinical symptoms and remission allows a subsequent reduction in the required dose of corticosteroids. The use of ECP in BP is poorly investigated: only one study [4] has reported a response in drug-resistant BP using a short-time ECP protocol.…”

“…Pediatric cases of BP are rare. Standard care entails oral steroid treatment; the effectiveness of the addition of plasma exchange (PEX) has not been established [1], even though it has been shown to allow the reduction of corticosteroid doses and to improve symptoms in therapy-resistant cases [2,3]. Short-term extracorporeal photochemotherapy (ECP) can provide effective and safe adjuvant treatment for patients with drug-resistant BP [4].…”

Pemfigusta Konvansiyonel Olmayan Tedavi Yaklaşımları

“…Mazzi ve arkadaşlarının yaptığı pemfigus vulgaris ve büllöz pemfigoid bulunan 5 hastadan oluşan bir çalışmada ortalama 10 plazmaferez işlemi ile tüm hastalarda tam remisyon sağlanmış ve steroid dozu %70'e kadar düşülebilmiştir (11). Turner ve arkadaşlarının yaptığı başka bir çalışmada ise immünsüpresif tedaviye rağmen yeterli yanıt alınamayan 7 hastada ortalama 5 plazmaferez işlemi ile 4 hastada tam remisyon, 2 hastada kısmi remisyon sağlanmışken 1 hastada etkisiz bulunmuştur (12).…”