Clinical Trial Evaluating Rituximab in Ocular Cicatricial Pemphigoid

Cicatricial pemphigoid is an autoimmune blistering disease which can affect the skin, mucous
membranes, and, in a small subset of patients, the eyes. Progressive ocular disease can lead
to irreversible damage and blindness. Conventional treatments have included high dose
systemic steroids, dapsone, and immunosuppressive agents such as azathioprine, methotrexate,
cyclophosphamide, and mycophenolate mofetil. However, there are a subgroup of patients who
fail to respond to these treatments, develop intolerable side effects, or have
contraindications to their use. Patients may also develop resistance to these conventional
treatment modalities. For these reasons, alternative treatment modalities are needed.
Rituximab has been very effective in the treatment of other autoimmune disorders. It has
recently been shown to be effective in the treatment of another autoimmune blistering
disorder known as pemphigus. We thus propose that Rituximab will be effective in the
treatment of cicatricial pemphigoid.

Inclusion Criteria:

1. Clinical diagnosis of ocular cicatricial pemphigoid (symptoms of conjunctivitis,
irritation, burning, increased lacrimation, photophobia, dryness of the eyes along
with conjunctival inflammation, trichiasis, and scarring

2. One of the following:

- Failed response to the use of one or more conventional treatments for a minimum
of 10 weeks; or

- Minimal conventional medication doses, with a significant adverse effects,
contradiction to use, or progressive disease despite treatment

3. Adults age 19 and older

4. Adequate renal function as indicated by serum creatinine levels less than 1.5

Exclusion Criteria:

1. known hypersensitivity to rituximab or its components

2. Age less than 19 years

3. Any other condition deemed by the investigator to be a significant hazard to the
subject if the investigational therapy were initiated.