Pemphigus is a severe autoimmune blistering disease mediated by circulating antibodies against certain proteins important for maintaining skin integrity. Protein A immunoadsorption is a dialysis-like technique selectively removing the antibodies from patient's blood. Rituximab is a synthetic antibody capable of destroying B cells. B cells are responsible for production of antibodies in the patients blood that, in turn, lead to clinical signs of pemphigus. Dexamethasone pulse therapy is a high-dose short-term corticosteroid therapy that may be used to suppress autoantibody production in pemphigus. While each of these three therapies had been used to treat pemphigus, none was shown effective in all cases. The hypothesis of this study is that a combination of protein A immunoadsorption, rituximab and dexamethasone is more effective that either of these treatments alone in achieving a rapid and durable improvement or cure in patients with pemphigus.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
23
Protein A Immunoadsorption: performed on 3 consecutive days every 3 weeks Rituximab: 1000 mg i.v. given twice at a 2-week interval Dexamethasone pulse therapy: 100 mg i.v. given on 3 consecutive days every 3 weeks Azathioprine: 2.5 mg/kg body weight daily p.o.
Department of Dermatology, University of Luebeck
Lübeck, Schleswig-Holstein, Germany
Number of Patients Achieving a Short- and Long-term Remission of Pemphigus
Clinical remission was graded as partial remission on therapy, complete remission on therapy and complete remission off therapy, as described by Murell et al, J Am Acad Dermatol, 2008; 58:1043-6.
Time frame: up to 43 months
Number of Patients Who Experienced Side-effects of Treatment
Patients who experienced side-effects were counted. In addition, the nature and severity of side-effects were recorded.
Time frame: up to 43 months
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