Long-term results of rituximab-intravenous immunoglobulin combination therapy in patients with epidermolysis bullosa acquisita resistant to conventional therapy
JOURNAL OF DERMATOLOGICAL TREATMENT, cilt.28, sa.1, ss.50-54, 2017 (SCI-Expanded, Scopus)
- 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.