Immunoadsorption, Dexamethasone Pulse Therapy and Rituximab for Pemphigus

Combined Treatment of Autoimmune Bullous Diseases With Protein A Immunoadsorption, Dexamethasone Pulse Therapy and Rituximab

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.

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.

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.

Patients who experienced side-effects were counted. In addition, the nature and severity of side-effects were recorded.

Inclusion Criteria: Diagnosis of pemphigus confirmed by immunofluorescence and desmoglein ELISA. Severe disease or past treatment(s) not effective or past treatment(s) not tolerated. Exclusion Criteria: General condition too poor to tolerate immunoadsorption treatment. Severe dementia or psychiatric disease.