A case of refractory Mooren's ulcer successfully treated with systemic immunosuppressive therapy

We will report the efficacy and precautions for the systemic administration of immunosuppressants in refractory Mooren's ulcer. A 65-year-old woman with refractory Mooren's ulcer was referred to our hospital. Initial visual acuity was 0.01 (logMAR 2.0) in the right eye and hand motion vision in the left eye. The right eye showed anterior chamber hemorrhage and a circumscribed peripheral ulcer with corneal perforation. The patient underwent conjunctival excision and lamellar keratoplasty in the right eye. Systemic methylprednisolone and oral cyclosporine (CsA) were initiated, along with topical betamethasone and tacrolimus. Systemic medications were continued with gradual tapering. At 8.5 months, the patient developed varicella-zoster virus (VZV)-associated keratouveitis in the right eye. Systemic steroid administration was promptly discontinued, but CsA was continued at a reduced dose. Antiviral treatment led to prompt improvement. Oral CsA was discontinued after 16 months of treatment. The total cumulative dose of PSL was 1,803 mg, and CsA was 64,730 mg. No relapses occurred during 36 months of follow-up. Multidisciplinary care was required to manage systemic complications. The long-term systemic administration of steroids and immunosuppressants may be associated with sustained remission in a patient with refractory Mooren's ulcer with appropriate monitoring for various systemic conditions caused by immunosuppression.