title: Low-dose pulsed intravenous cyclophosphamide for severe ocular cicatricial pemphigoid in elderly patients.
abstract: OBJECTIVE
  Ocular cicatricial pemphigoid (OCP) is a relatively rare autoimmune disease affecting elderly patients and causing severe symptoms that may culminate in blindness. Treatment is based on immunosuppression, but optimal regimens have not been established.
  
  
  METHODS
  A prospective unmasked case series of all patients with severe OCP who gave consent and completed treatment and ≥6 months of follow-up in the cornea and immunomodulation outpatient clinics of an academic hospital. Monthly pulses of intravenous cyclophosphamide (IVC; 500 mg) were administered with ondansetron and adjusted according to response.
  
  
  RESULTS
  Over 14 years, 13 patients (median age 77 years) met inclusion criteria. All had bilateral OCP (2 eyes were previously blind), but extraocular involvement was rare (1/13). Three to 28 pulses were given, and the patients were followed up for a median of 32 (range, 6-167) months. Remission of inflammation in both eyes was achieved in 12 patients (92%). Vision improved in 5 of 13 patients, stabilized in another 5 (combined, 77%), and worsened in only 3 patients. One patient's condition flared up during treatment that responded to steroids and increasing IVC frequency. In 4 patients and 6 eyes (25%), cicatrization progressed (usually, Foster stages 1-3). Two late relapses occurred and responded to retreatment. IVC was generally well tolerated, although nausea led to modification in 2 patients. One patient developed candida keratitis.
  
  
  CONCLUSIONS
  Compared with other treatment modalities, low-dose monthly pulse IVC is found to be a relatively safe, simple, and usually effective alternative immunosuppressive treatment in severe OCP.
authors.affiliation: *Departments of Medicine and †Ophthalmology, Kaplan Medical Center, Rehovot, Hebrew University Hadassah Medical School, Jerusalem, Israel.
authors.forename: Joshua
authors.initials: J
authors.lastname: Friedman
authors.forename: Arie L
authors.initials: AL
authors.lastname: Marcovich
authors.forename: Guy
authors.initials: G
authors.lastname: Kleinmann
authors.forename: Ami
authors.initials: A
authors.lastname: Schattner
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000932:Antiemetics / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007275:Injections, Intravenous; D008297:Male; D008875:Middle Aged; D017294:Ondansetron / Q000627:therapeutic use; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000503:physiopathology; D011446:Prospective Studies; D020551:Pulse Therapy, Drug
chemical_list: D000932:Antiemetics; D007166:Immunosuppressive Agents; D017294:Ondansetron; D003520:Cyclophosphamide