Rituximab in autoimmune bullous diseases: mixed responses and adverse effects.
BACKGROUND
Intolerably high doses of systemic corticosteroids and additional immunosuppressants may be required to control disease activity in autoimmune bullous skin diseases. New therapeutic options are needed for such patients.


OBJECTIVE
To determine the efficacy and adverse effects of adjuvant rituximab.


METHODS
Seven patients with refractory autoimmune blistering diseases (pemphigus vulgaris, PV, n = 4; bullous pemphigoid, BP, n = 2; mucous membrane pemphigoid, MMP, n = 1) were treated four times with rituximab at an individual dose of 375 mg m(-2) at weekly intervals.


RESULTS
All lesions cleared in three patients (two PV, one BP), while they were reduced by more than 50% in three others (two PV, one BP). The concomitant immunosuppressive medication was reduced in five patients (four PV, one BP). The patient with MMP developed bilateral blindness while nasopharyngeal lesions resolved. Three patients (two BP, one PV) experienced severe adverse events including fatal pneumonia.


CONCLUSIONS
Adjuvant B-cell depletion by rituximab is effective in otherwise therapy-resistant bullous autoimmune disorders but may be associated with substantial adverse effects including fatal outcomes.
Schmidt
E
E
Department of Dermatology, University of Würzburg, Josef-Schneider-Strasse 2, D-97080 Würzburg, Germany.schmidt_e@klinik.uni-wuerzburg.de
Seitz
C S
CS
Benoit
S
S
Bröcker
E B
EB
Goebeler
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000911:Antibodies, Monoclonal / Q000008:administration & dosage* / Q000009:adverse effects; D058846:Antibodies, Monoclonal, Murine-Derived; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000188:drug therapy*; D009092:Mucous Membrane; D009668:Nose Diseases / Q000188:drug therapy*; D010392:Pemphigus / Q000188:drug therapy*; D000069283:Rituximab; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab