Clinical and immunological follow-up of pemphigus patients on adjuvant treatment with immunoadsorption or rituximab.
BACKGROUND
Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease which is associated with pathogenic IgG autoantibodies against desmogleins (Dsg) 1 and 3. Novel therapeutic strategies such as immunoadsorption (IA) or the anti-CD20 antibody rituximab (Rtx) hold promise to be effective in severe or recalcitrant PV.


METHODS
In the present retrospective study, 6 patients with extensive cutaneous PV were subjected to adjuvant IA treatment while 5 patients with severe mucosal PV received adjuvant Rtx treatment.


RESULTS
Within 6 months, IA and Rtx induced excellent clinical responses which were associated with a significant reduction of prednisolone doses and a decrease in anti-Dsg-specific IgG. Over a 12-month period, 3 IA-treated patients required additional adjuvant drugs while all of the PV patients on Rtx had no or only minimal residual symptoms.


CONCLUSIONS
The relative therapeutic (long-term) efficacy of IA and Rtx in cutaneous versus mucosal PV needs to be evaluated in a prospective study.
Pfütze
Martin
M
Department of Dermatology and Allergology, Philipps University, Marburg, Germany.
Eming
Rüdiger
R
Kneisel
Andrea
A
Kuhlmann
Uwe
U
Hoyer
Joachim
J
Hertl
Michael
M
D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood; D051183:Desmoglein 1 / Q000276:immunology; D051184:Desmoglein 3 / Q000276:immunology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D007155:Immunologic Factors / Q000627:therapeutic use*; D007163:Immunosorbent Techniques; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D000069283:Rituximab
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D051183:Desmoglein 1; D051184:Desmoglein 3; D007074:Immunoglobulin G; D007155:Immunologic Factors; D000069283:Rituximab