Adjuvant therapy of severe and/or refractory bullous pemphigoids with immunoadsorption by LIGASORB®, dapsone, prednisolone and topical mometasone fumarate (LiBPem-Study).
- Conditions
- L12.0Bullous pemphigoid
- Registration Number
- DRKS00013775
- Lead Sponsor
- niversitätsklinikum Schleswig-Holstein Campus Lübeck
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Recruiting
- Sex
- All
- Target Recruitment
- 10
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))
- 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
Study & Design
- Study Type
- observational
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method umber and degree of severity (1-5, according to the standard criteria of the WHO) of adverse events within study duration (12 months)<br><br>
- Secondary Outcome Measures
Name Time Method -time to complete remission under therapy, defined as complete remission of BP lesions<br>-Duration of complete Remission under therapy<br>-Number of Patients in Remission, 6 and 12 months post immunoadsorption<br>-decrease of Anti-BP180-seraautoimmunantibodies, 1, 3, 6, and 12 months post immunoadsorption<br>-reduction of the clinical disease activity scores (Bullous Pemphigoid Disease Area Index ; BPDAI), 3, 6, 12 months post immunoadsorption<br>-number of patients with doses of prednisolon <7.5 mg/d (threshold for the cushing-syndrome) 6 and 12 months post immunoadsorption<br>-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