B Horváth1, J Huizinga, H H Pas, A B Mulder, M F Jonkman. 1. Departments of Dermatology Laboratory Medicine, Centre for Blistering Diseases, University Medical Centre Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, the Netherlands. b.horvath@umcg.nl
Abstract
BACKGROUND: Rituximab, an anti-CD20 antibody, was shown in open series studies to be effective in treating pemphigus at a dose of 4 × 375 mgm(-2) as approved for B-cell malignancies. OBJECTIVES: We investigated whether a lower dose of rituximab is also effective for pemphigus. METHODS: Patients with pemphigus were treated with a single course of two infusions of rituximab (500 mg each) at an interval of 2 weeks. Clinical consensus late end points, B-cell number, desmoglein 1 and desmoglein 3 indices were monitored. RESULTS: We enrolled 15 patients in the study: three with pemphigus foliaceus (PF) and 12 with pemphigus vulgaris (PV). The follow-up was 32-152 weeks (median 94). All 15 patients responded to therapy. Eight patients achieved complete remission in a median period of 5 weeks (four on minimal therapy, four off therapy). Seven patients achieved partial remission in a median period of 34·5 weeks (five on minimal therapy, two off therapy). Relapses (40%) were seen between 53 and 103 weeks (median 97) after start of therapy. B-cell numbers dropped to <1% after first infusion, and remained undetectable in patients with sustained remission. The antidesmoglein 1 index correlated well with the clinical severity in PF, but this was less obvious in PV. CONCLUSIONS: A low dose of rituximab is an effective and safe treatment for pemphigus. Relapses may occur, mostly at the end of the second year. Cost-effectiveness studies with a long follow-up are required to determine the proper dosage of this expensive drug in pemphigus.
BACKGROUND:Rituximab, an anti-CD20 antibody, was shown in open series studies to be effective in treating pemphigus at a dose of 4 × 375 mgm(-2) as approved for B-cell malignancies. OBJECTIVES: We investigated whether a lower dose of rituximab is also effective for pemphigus. METHODS:Patients with pemphigus were treated with a single course of two infusions of rituximab (500 mg each) at an interval of 2 weeks. Clinical consensus late end points, B-cell number, desmoglein 1 and desmoglein 3 indices were monitored. RESULTS: We enrolled 15 patients in the study: three with pemphigus foliaceus (PF) and 12 with pemphigus vulgaris (PV). The follow-up was 32-152 weeks (median 94). All 15 patients responded to therapy. Eight patients achieved complete remission in a median period of 5 weeks (four on minimal therapy, four off therapy). Seven patients achieved partial remission in a median period of 34·5 weeks (five on minimal therapy, two off therapy). Relapses (40%) were seen between 53 and 103 weeks (median 97) after start of therapy. B-cell numbers dropped to <1% after first infusion, and remained undetectable in patients with sustained remission. The antidesmoglein 1 index correlated well with the clinical severity in PF, but this was less obvious in PV. CONCLUSIONS: A low dose of rituximab is an effective and safe treatment for pemphigus. Relapses may occur, mostly at the end of the second year. Cost-effectiveness studies with a long follow-up are required to determine the proper dosage of this expensive drug in pemphigus.
Authors: Christoph T Ellebrecht; Eun J Choi; David M Allman; Donald E Tsai; William A Wegener; David M Goldenberg; Aimee S Payne Journal: JAMA Dermatol Date: 2014-12 Impact factor: 10.282
Authors: Mina Kalantari-Dehaghi; Grant J Anhalt; Michael J Camilleri; Alex I Chernyavsky; Sookhee Chun; Philip L Felgner; Algis Jasinskas; Kristin M Leiferman; Li Liang; Steve Marchenko; Rie Nakajima-Sasaki; Mark R Pittelkow; John J Zone; Sergei A Grando Journal: PLoS One Date: 2013-03-07 Impact factor: 3.240