Efficacy of B cell-targeted therapy in autoimmune bullous diseases

• Conditions: autoimmune bullous diseases; blistering diseases; 10014982

• Registration Number: NL-OMON51059
• Lead Sponsor: niversitair Medisch Centrum Groningen

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Last Update Posted: 9 April 2024

Recruitment & Eligibility

• Status: Pending
• Sex: Not specified
• Target Recruitment: 200

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

• Patients under the age of 18 years.
• Plasma exchange, plasmapheresis or immunoadsorption within the last 3 months

Study & Design

• Study Type: Observational invasive
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>B-cell phenotype, B-cell repertoire, including number of expanded B-cell clones</p><br>