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A Journal on Ophthalmology




Minerva Oftalmologica 2011 September;53(3):57-61


language: English

Rapidly-progressive neuropapillitis in a patient with Behçet’s disease: successful treatment with intravenous cyclophosphamide

Grassi P. 1, Naclerio C. 2, Lanzotti A. 1

1 Unit of Internal Medicine, Scarlato Hospital, Scafati, Salerno, Italy 2 Unit of Rheumatology, “Scarlato” Hospital, Scafati, Salerno, Italy


A 38-year-old woman presented with complete loss of vision in the right eye in ten days. Visual acuity was 2/10 in the right eye and 4/10 in the left eye. Examination of eyes showed a papillary defect more marked of the optic nerve heads, fundus examination showed blurred disc margins. Visual evoked potentials (VEP) on the right side were prolonged. She also presented recurrent oral aphtosis, skin pustular lesions and erythema nodosum. We diagnosed optic neuritis secondary to Behçet’s disease (BD) and started treatment with intravenous methylprednisolone at a dose of 1g daily for three days monthly for six months. Clinical response was noted in seven days with visual acuity returning to 6/10 and a partial resolution of papillitis on ophthalmoscopy was observed. At follow-up 3 months later, visual acuity was 4/10 in the right eye and 5/10 in the left eye. On ophthalmoscopy examination there was a recidive of papillitis. Cyclophosphamide at a dose of 1g monthly was started. After 1 month, she presented a visual acuity of 7/10 in the right eye and 8/10 in the left eye. At this point, intravenous monthly cyclophosphamide pulse therapy was undergone. After sixth pulse therapy, visual acuity was well preserved. This response was probably due to the fact that BD in our patient was in the active phase. A systematic study of cyclophosphamide therapy in the treatment of “acute” BD must be carried out to assess its role in the ocular BD treatment.

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