Conditions: Pemphigus
Keywords: pemphigus rituximab immunoadsorption dexamethasone treatment management
Allocation: NA | Intervention model: SINGLE_GROUP
Enrollment: 23
Healthy volunteers: no

Brief summary:
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.

Detailed description:

Arms:
arm 1: None

Interventions: intervention 1: Combination of Protein A Immunoadsorption, Rituximab, Dexamethasone plus Azathioprine
Intervention descriptions: intervention 1: 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.

Protocol document: