Case 26-1

A 38-year-old Korean man had a 2-year history of symptoms supporting the diagnosis of Behçet’s disease. He had had biopsy-proved erythema nodosum, as well as several episodes of painful and tender joints, including the knee, ankle, and foot. He had several mouth ulcers present simultaneously, as well as a penile ulcer. He showed HLA-B51 positivity. For the past year he had noticed that the vision in his left eye was blurry. Attempts were made to treat the condition with eyedrops, but the problem appeared to wax and wane despite therapy. The patient seemed to have on average an attack only in the left eye once a month. When we first saw him, visual acuity was 20/12.5 in the right eye and 5/125 in the left.

The right eye showed no evidence of current or prior uveitic episodes. The left eye had trace evidence of flare and cells in the anterior segment. There were 3% cells and 2% haze in the vitreous. The retina had areas of gliosis, with diffuse sheathing and markedly attenuated retinal vessels. Because the patient had unilateral disease and a recent recurrence was resolving, he was given a regimen of 0.6 mg colchicine three times daily. He was able to tolerate this dose schedule, and repeated complete blood cell counts showed no alterations. The number of ocular attacks in the left eye decreased from once a month to one every 7–9 months. He has continued this treatment regimen.

Case 26-2

A 39-year-old man of Scottish-Irish ancestry had a 6-year history of arthritis, mouth ulcers, acne-like lesions on his back and penile ulcers. In addition, recurrent anterior and posterior uveitis developed 2 years before he was seen by us. These episodes were treated initially with systemic steroids, but because of

the increasing frequency of ocular attacks he was given chlorambucil. This regimen was maintained for 6 months, with some reduction in the number of ocular attacks. However, his white blood cell count began to decrease to < 3000/mm3. This

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Case 26-3

A 35-year-old man of Greek extraction developed all four major criteria for Behçet’s disease within the span of 1.5 years. Initial therapy with chlorambucil and then cyclophosphamide (Cytoxan) resulted in some reduction in his retinal vasoocclusive attacks. The best-corrected vision decreased to 20/80 OD and 20/30 OS. Fluorescein angiographic examination showed marked areas of capillary dropout, and there was some retinal atrophy, with the optic nerve in the right eye beginning to look pale. The patient was given ciclosporin therapy and a marked resolution of his nonocular symptoms was seen; his ocular attacks dissipated. Over a period of 3 years his vision dropped markedly in both eyes despite the absence of inflammatory disease. When he returned several years later, his vision was reduced to hand movements OD and 20/400 OS. His therapy was stopped.

Comment 26-3.  This patient presents a most frustrating scenario. It would seem that once retinal alterations due to the uveitis have progressed extensively, the degenerative changes become autonomous of the inflammatory disease that initiated them. We assume that apoptosis – programmed cell death – has been initiated. Once this has occurred, vision will irreversibly decrease, even if the uveitis has been controlled.

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