L12.0
Conditions
Interventions
Group 1: Immunoadsorption (IA) is conducted on 3 consecutive days (day 1, 2, 3). In case of relapse with highly elevated autoantibody levels, an additional 3-day- IA-cycle can be conducted.The combina
Sponsors
Universitätsklinikum Schleswig-Holstein Campus Lübeck
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: - Patiens with diagnostically confirmed BP (direct immunofluorescence, proof of IgG-reactivity against PB180 in ELISA) - Age >= 18 years of Age - written informed consent - > 30 % of Body surface area with BP-lesions OR - refractive therapy to conventional immunosuppression / immunomodulation according to the Guidelines of the German Dermatology Society (Deutsche Dermatologische Gesellschaft (Eming et al., j Dtsch Dermatol Ges 2015))
Exclusion criteria
Exclusion criteria: - proven oversensitivity against materials and/or medications used in the study (see allergy pass) - severe coagulopathy - severe cardiovascular disease (NYHA IV, myocardial infarction within the last 3 months) - intake of ACE-Inhibitors - women of child-bearing age who do not use adequate birth control methods - severe systemic infection (HBsAg-positive chronic active Hepatitis B, Hepatitis C, HIV-infection, florid TBC-infection, acute viral infections (e.g., variella, Zoster Virus, severe HSV-1 infection) - pregnant or lactating women - severe congenital immune deficiency - florid gastro-duodenal ulcer - active, progressive malignomas, or malignomas which are currently treated with chemo- / immunotherapy. Patients with malignomas in complete Remission have to consult an oncologist Prior to study enrollment -severe liver and kidney dysfunction (>= 3 times elevated serum-GOT, >= 3 times elevated Serum creatinine - Hb < 9g/dl or leukopenia < 3000/microliter or thrombocytopenia < 100.000/mircoliter with reduced bone marrow function - acute or unstable psychiatric diseases with high risk of exacerbation with intake of high-dose prednisolone - written informed consent not possible - inadequte knowledge of the German language or illiteracy (questionnaires have to be filled out by the study Patients themselves) - concurrent participation in another clinical trial
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number and degree of severity (1-5, according to the standard criteria of the WHO) of adverse events within study duration (12 months) | — |
Secondary
| Measure | Time frame |
|---|---|
| -time to complete remission under therapy, defined as complete remission of BP lesions -Duration of complete Remission under therapy -Number of Patients in Remission, 6 and 12 months post immunoadsorption -decrease of Anti-BP180-seraautoimmunantibodies, 1, 3, 6, and 12 months post immunoadsorption -reduction of the clinical disease activity scores (Bullous Pemphigoid Disease Area Index ; BPDAI), 3, 6, 12 months post immunoadsorption -number of patients with doses of prednisolon <7.5 mg/d (threshold for the cushing-syndrome) 6 and 12 months post immunoadsorption -number of patients with a Dermatology Life Quality Index (DLQI) score of 2-5 (= small effect on Quality of life), 3, 6, and 12 months post immunoadsorption | — |
Countries
Germany
Contacts
Public ContactHeike Kross
Klinik für Dermatologie, Allergologie und VenerologieUniversitätsklinikum Schleswig-Holstein Campus Lübeck
Outcome results
None listed