Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid: a preliminary study.
OBJECTIVE
To report the effects of intravenous immunoglobulin treatment of ten patients with progressive ocular cicatricial pemphigoid who did not respond to conventional immunomodulatory regimens.


METHODS
Noncomparative, interventional case series.


METHODS
Ten patients with biopsy-proven progressive cicatricial pemphigoid affecting the eyes who did not respond adequately to other local and systemic immunosuppressive treatment regimens.


METHODS
Intravenous infusions of pooled human immunoglobulin, 2 to 3 g/kg body weight/cycle, divided over 3 days, and repeated every 2 to 6 weeks.


METHODS
Reduction in conjunctival inflammation, prevention of progression of subepithelial conjunctival fibrosis, improvement in ocular symptoms (discomfort, photophobia), improved visual acuity, reduction in extraocular mucosal lesions.


RESULTS
Clinical deterioration was arrested and resolution of chronic conjunctivitis was documented in all ten patients. Maximum therapeutic effect was observed and maintained after a minimum of 4 cycles of therapy; three patients required 12 cycles before disease control. The duration of therapy in these ten patients has been 16 to 23 months (mean, 19.3 months) with no treatment-induced side effects. Extraocular mucosal lesion resolution has occurred in all but one patient, Visual acuity has stabilized or improved in all ten patients, and subjective complaints of discomfort and photophobia have decreased in all patients.


CONCLUSIONS
Intravenous immunoglobulin immunomodulatory therapy can be a safe and effective therapy for otherwise treatment-resistant ocular cicatricial pemphigoid.
Foster
C S
CS
Uveitis and Immunology Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114, USA. fosters@helix.mgh.harvard.edu
Ahmed
A R
AR
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000276:immunology; D002908:Chronic Disease; D003228:Conjunctiva / Q000473:pathology; D003231:Conjunctivitis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D005260:Female; D005355:Fibrosis; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D039663:Integrin beta4; D016023:Integrins / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome; D014792:Visual Acuity
D015703:Antigens, CD; D016756:Immunoglobulins, Intravenous; D039663:Integrin beta4; D016023:Integrins