Role of cyclophosphamide and high dose steroid in ocular cicatricial pemphigoid.

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Abstract
AIMS/BACKGROUND--Ocular cicatricial pemphigoid (OCP) can present with severe conjunctival inflammation that requires systemic immunosuppression to avoid serious ocular morbidity. This study aimed to assess the clinical response to cyclophosphamide and short term, high dose prednisolone in this group of patients. METHODS--A prospective, unmasked study assessed patients presenting with either 'severe' ocular inflammation (n = 4) or 'marked' or 'severe' ocular inflammation that had failed to respond to other systemic immunosuppression (n = 6). Nineteen inflamed eyes of 10 consecutive patients were enrolled. RESULTS--The ocular inflammation resolved in 15 eyes in a mean time of 2.4 months. Two eyes perforated despite treatment and one patient was unable to tolerate the medication. Progressive cicatrisation occurred in 21%. CONCLUSION--Cyclophosphamide and short term, high dose prednisolone are effective in severe inflammation caused by OCP but may not completely prevent cicatrisation.