Plasmapheresis/immunoadsorption in patients with pemphigus

Background and Design: Plasmapheresis or immunoadsorption which is a more advanced method are among the treatment options that induce a rapid remission in severe and resistant pemphigus. The aim of this study is to evaluate the efficacy and safety of plasmapheresis or immunoadsorption therapies via the clinical experience gained from our patients with pemphigus vulgaris and paraneoplastic pemphigus.
Materials and Methods: Among patients who were diagnosed with pemphigus according to the histopathology and/or immunofluorescence methods and followed-up at our bullous diseases outpatient clinic between the years 1987 and 2013, 7 patients (5 PV, 2 PNP), who were treated with plasmapheresis or immunoadsorption, were retrospectively analyzed. Among these patients, 3 were treated with classical plasmapheresis, whereas 2 with double-filtration plasmapheresis, 1 with immunoadsorption, and 1 initially with classical plasmapheresis followed by immunoadsorption due to impairment of the hemodynamic parameters. The clinical findings, antibody titers, and immunological responses before and after the therapy, the therapy dosages and adverse effects were evaluated according to the data gained from the patient files.
Results: In 4 patients (1., 3., 4. and 5. patients) with pemphigus vulgaris, complete clinical response was achieved, while 1 patient (2. patient) showed partial regression in skin lesions. In both of the patients (6.,7. patients) with paraneoplastic pemphigus the therapy discontinued due to complications. In all of the evaluated patients, the analysis of the antibody titers before and after the therapy revealed lower levels in comparison to those detected at the initiation of the therapy. In 2 patients hypoalbuminemia and in 2 patients catheter infection were developed as the side effects of the therapy.
Conclusion: Even though plasmapheresis or immunoadsorption may be chosen as a good mid-treatment method to decrease the severity of the disease especially in resistant patients with pemphigus, it must be borne in mind that because of the exacerbations that may be seen after the treatment, these therapies should be used in combination with immunosuppressives, and are not effective in the long-term.

Pemfiguslu hastalarda plazmaferez/immünadsorbsiyon sonuçlarý

Amaç: Þiddetli ve tedaviye dirençli pemfigus hastalýðýnda plazmaferez ve daha geliþmiþ bir yöntem olan immunadsorpsiyon hýzlý remisyon saðlayan tedavi yöntemleri arasýndadýr. Pemfigus vulgaris ve paraneoplastik pemfiguslu hastalarýmýzda plazmaferez veya immunadsorpsiyon tedavisi uygulanmýþ olgulardan edindiðimiz klinik deneyim ile bu tedavilerin etkinliði ve yan etkilerinin deðerlendirilmesi amaçlanmýþtýr.
Gereç ve Yöntem: Büllü hastalýklar polikliniðinde 1987-2013 yýllarý arasýnda takip edilen histopatoloji ve/veya immunfloresan yöntemlerle pemfigus tanýsý konulan hastalar arasýndan plazmaferez veya immunadsorpsiyon tedavisi uygulanan 7 hasta (5’i PV, 2’si PNP) retrospektif olarak deðerlendirildi.Bu hastalardan üç üne klasik plazmaferez, 2 hastaya double filtrasyon plazmaferez, 1 hastaya immunadsorbsiyon; 1 hastaya önce klasik plazmaferez, hemodinamik parametrelerde bozukluk geliþmesi üzerine sonrasýnda immunadsorbsiyon tedavisi uygulanmýþtý.Hastalarýn tedavi öncesi ve sonrasý klinik bulgularý, antikor titreleri ile immunolojik yanýtlarý,tedavi dozlarý ve yan etkileri, takip dosyalarýndan edinilen bilgilere göre deðerlendirmeye alýndý.
Bulgular:
Pemfigus vulgaris’li hastalardan 4 ’ünde (1,3,4,5. hastalar) tam klinik yanýt, 1 hastada (2. hasta) deri lezyonlarýnda kýsmi gerileme görüldü. Paraneoplastik pemfigus tanýsýyla izlenen 2 hastada (6. ve 7. hastalar) geliþen komplikasyonlar nedeniyle tedavi sonlandýrýldý. Tedavi öncesi ve sonrasý yapýlan dolaþan antikor ölçümlerinde, deðerlendirme yapýlan hastalarýn tamamýnda tedavi sonrasý antikor titresi baþlangýca göre düþük saptandý. Yan etki olarak 2 hastada hipoalbuminemi, 2 hastada katater infeksiyonu geliþti.
Sonuç:
Plazmaferez veya immunadsorbsiyon,özellikle tedaviye dirençli pemfiguslu hastalarda hastalýðýn þiddetini azaltacak iyi bir ara tedavi yöntemi olarak seçilebilirse de, tedavi sonrasýnda alevlenme görülebileceði,bu nedenle immunsupresifler ile birlikte kullanýlmasýnýn gerektiði ve uzun dönem için etkili olmadýðý hatýrda tutulmalýdýr.