autoimmune bullous diseases blistering diseases
Conditions
Interventions
None listed
Sponsors
Universitair Medisch Centrum Groningen
Eligibility
Age
18 Years to 64 Years
Inclusion criteria
Inclusion criteria: • Age of 18 years or older. • Diagnosis of pemphigus, based on the following criteria: clinical features suggestive of pemphigus vulgaris or pemphigus foliaceus; a histological image of intraepidermal acantholysis; and/or deposition of IgG, complement component 3, or both on the keratinocyte membrane detected by direct immunofluorescence • Diagnosis of pemphigoid, based on the following criteria: linear depositions of IgG, IgA, IgM, or C3c along the BMZ by direct immunofluorescence microscopy (DIF) and/or positive indirect immunofluorescence microscopy (IIF) on salt-split skin (SSS), in combination with clinical presentation, histopathological findings, or other immunoserological tests compatible with the diagnosis of a pemphigoid disease. • Administration of rituximab according to the following protocol: two infusion of 1000mg within an interval of two weeks, followed by 500mg at month 6 and month 12, or in patients with severe pemphigus 1000mg at month 6, or in patients who are not in complete remission two infusions of 1000mg two weeks apart.
Exclusion criteria
Exclusion criteria: • Patients under the age of 18 years. • Plasma exchange, plasmapheresis or immunoadsorption within the last 3 months
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| B-cell phenotype, B-cell repertoire, including number of expanded B-cell clones | — |
Secondary
| Measure | Time frame |
|---|---|
| Laboratory serum and cellular parameters: • Antigen-specific B cell receptor sequences • RNA expression profile of antigen-specific B-cells • Peripheral blood mononuclear cells (PBMC*s) • Free light chains (FLCs) as a biomarker for plasmacells • Anti-desmoglein 1 and 3 antibodies • Antibodies against the noncollagenous 16A domain of BP180 (NC16A) and BP230 • Serum B-lymphocytes, T-lymphocytes • Serum total IgG and subclasses • Glycosylation status and binding characteristics of IgG antibodies Clinical parameters: • Response outcomes were defined according to international consensus and measured by the early endpoint disease control (DC), and the late endpoints partial remission (PR), complete remission (CR), and the number of relapses. • Pemphigus Disease Area Index (PDAI) • Bullous Pemphigoid Disease Area Index (BPDAI) • Mucous Membrane Pemphigoid Disease Area Index (MMPDAI) • Adjuvant immunomodulatory or immunosuppressive treatment • Dermatology Life Quality Index (DLQI) • Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) • Visual Analogue Scale (VAS) pain and itch • Geriatric 8 (G-8) Score in patients above 50 years | — |
Countries
Netherlands
Outcome results
None listed