In all 24 patients acute manifestations of ocular involvement resolved within one week (Figs. 4 and 5). The treatment was effective against vasculitis (n=21), iritis (n=20) and neuropathy of the optic nerve (n=12). Five patients showed a recurrence. Among them, one patient stopped treatment on her own (Fig. 3), another one because of pregnancy. One patient had seven recurrences under treatment, however he also presented recurrences under cyclosporine A, chlorambucil, and tumour necrosis factor-a antagonist etanercept. In four patients we were able to discontinue treatment without any recurrence for a year.
Constant visual acuity was reached in 10 patients (42%), 11 patients (46%) showed a better (2-3 lines) visual acuity as compared to the acute period of inflammation. In three patients (13%) visual acuity decreased because of the development of cataract and neuropathy. Altogether, a constant or improved vision was achieved in 88% of all patients (21 out of 24 patients).
3.1 Case report
One of the patients underwent repeated intraocular surgery because of complications due to his Behget's disease. He was 24 years old when he was introduced to us in 1999. He was of Turkish origin and had a visual loss in his right eye. Adamantiades-Behget's disease has been diagnosed two years before. Arthritis of the knees, oral aphtosis, and skin lesions, manifesting as folliculitis were his first symptoms. At that time the treatment was a monotherapy with 10 mg/d prednisolone per os. Three months before he was sent to us he noticed a loss of vision in his right eye. He had a hypopyon-iritis, and a retinal detachment was suspected.
At the initial presentation visual acuity was light perception on his right eye and 20/20 on his left eye. On his right eye the cornea was clear, anterior chamber deep with cells and tyndall positive. The iris showed a rubeosis and posterior synechiae (Fig. 6). Funduscopy and ultrasound examination showed a total retinal detachment (Fig. 7). On his left eye the anterior chamber showed no signs of acute inflammation, but funduscopy showed inflamed and occluded vessels in the nasal periphery and snowballs in the lower periphery. The macular and the optic disks were without pathology. Intraocular pressure was 5 mmHg on the right and 35 mmHg on the left side.
General symptoms were oral aphthosis, arthralgia in his right knee, folliculitis, and a positive pathergy test. Family history was negative.
After the diagnosis has been confirmed a therapy as described before was started. After two weeks the acute inflammation disappeared. Quite remarkably, the rubeosis also decreased on his right eye (Fig. 8).
A vitrectomy was performed to save the right eye. Intraoperatively a total retinal detachment with an anterior traction, just behind the lens adhesive to the posterior lens capsule was detected and a 360° retinectomy and a lentectomy were performed. After removal of several epiretinal membranes, the central retina was reattached under a silicone oil tamponade (Fig. 9).
Following the operative treatment, a monotherapy with interferon-alpha-2a (3x9 Mill. IU/3xweek) was administered, and no recurrence of the disease occurred. The visual acuity was 20/200 on the right eye at that time.
On the left eye there was no possibility to treat the glaucoma conservatively. Gonioscopy showed neovascularisation in the anterior chamber angle. A panretinal laser photocoagulation of the left retina was performed. The neovascularisation decreased but the intraocular pressure was still increased, therefore a trabeculectomy was performed.
Postoperatively the tension is now under control without any additional medical treatment since three years (Fig. 10).
At present visual acuity is 20/200 on the right eye and 20/20 on the left eye. Since the begin of the interferon therapy, no relapse of the disease has occurred. The dose was lowered to 3 Mill. IU/3x week.
Flu-like symptoms occurred in all of our patients, tachycardia in one patient, and dose-dependent reversible thrombocytopenia and reversible alopecia were the only additional side-effects. The flu-like symptoms were well tolerated under the addition of non-steroidal antiphlogistics. Antinuclear antibodies were detected in two patients. Considering the risk of secondary ophthalmologic complications, these side effects have to be accepted.
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