Long-term results of rituximab-intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy

Oktem A., AKAY B. N., BOYVAT A., KUNDAKCI N., Erdem C., BOSTANCI S., ...Daha Fazla

JOURNAL OF DERMATOLOGICAL TREATMENT, cilt.28, sa.1, ss.50-54, 2017 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 28 Sayı: 1
  • Basım Tarihi: 2017
  • Doi Numarası: 10.1080/09546634.2016.1179711
  • Dergi Adı: JOURNAL OF DERMATOLOGICAL TREATMENT
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.50-54
  • Anahtar Kelimeler: Epidermolysis bullosa acquisita, rituximab, intravenous immunoglobulin, CLINICAL-RESPONSE, PEMPHIGUS, IMMUNOADSORPTION, RECALCITRANT, DISEASES
  • Ankara Üniversitesi Adresli: Evet

Özet

Background: Epidermolysis bullosa acquisita (EBA) is a rare subepidermal bullous disease. Long-term remission in this disease is difficult using current treatments, unlike that in patients with other autoimmune bullous diseases.Objective: We retrospectively evaluated the effectiveness and side effects of rituximab-intravenous immunoglobulin (IVIg) combination treatment in five patients with EBA resistant to conventional treatment.Patients and methods: Rituximab (375mg/m(2)) was administered for four consecutive weeks to four patients, and their treatment continued with IVIg at a dose of 2g/kg/month. One patient received two cycles of rituximab for three consecutive weeks, IVIg in the fourth week, followed by monthly IVIg administrations as in the other patients.Results: The total number of IVIg therapy cycles ranged from 10 to 26 (mean 19.4). Mean skin involvement, mucosal involvement, and disease severity scores decreased after a mean follow-up of 22.6 months (range, 10-28 months). In an analysis performed during months 24-28, the number of CD19-positive B cells was found to be below the normal reference range in four patients.Limitations: This was a retrospective study with a limited number of patients.Conclusion: Rituximab-IVIg combination treatment seems to be effective and safe for treating patients with EBA resistant to conventional treatments.