Clinical Trial Evaluating Rituximab in Ocular Cicatricial Pemphigoid

• Number of Participants With no Evidence of Further Scarring (Fosters Staging) at 16 Weeks [ Time Frame: 16 weeks ] [ Designated as safety issue: No ]

  Stages Characteristics I Subconjunctival scarring and fibrosis II Fornix foreshortening (a-d describes % loss of inferior fornix depth)

  1. 0-25%
  2. 25-50%
  3. 50-75%
  4. 75-100% III Presence of symblepharon and number (n) (a-d describes % of horizontal involvement by sympblephara and n is the number of symblephara countable)

  a. 0-25% b. 25-50% c. 50-75% d. 75-100% IV Ankyloblepharon, frozen globe

  
  
• 2. The Proportion of Patients That Experience a Grade 3, Grade 4, or Grade 5 Toxicity Based Reaction on the NCI-CTC System at the Time of Their Infusions and During Follow-up Visits. [ Time Frame: 16 weeks ] [ Designated as safety issue: No ]
  

• 1. Stability of Visual Acuity (Snellen's Test) at 16 Weeks [ Time Frame: 16 weeks ] [ Designated as safety issue: No ]
  
• 2. Stability of Visual Acuity (Snellen's Test) at 24 Weeks [ Time Frame: 24 weeks ] [ Designated as safety issue: No ]
  

Drug: Rituximab
The Rituximab dose is 1000mg (1gm) given as an IV infusion every two weeks for 2 doses (days 1 and 15).
Other Name: Rituximab

Cicatricial pemphigoid is an autoimmune blistering disease whcih 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.