Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease.

PURPOSE: To assess the outcome of retinal vasculitis in patients with Behçet disease treated with infliximab compared to treatment with conventional therapy. DESIGN: Nonrandomized, retrospective comparative clinical study. METHODS: Patients with Behçet disease with all four major criteria were included in this study. Patients had recurrent episodes of uveitis and retinal vasculitis. Thirty-three patients (Group 1) were treated with oral prednisone, cyclosporine, and azathioprine or methotrexate for a minimum period of three months. Ten patients (Group 2) who failed to respond to conventional therapy were given infliximab at a dose of 5 mg/kg in a single intravenous infusion on day 1 and every two weeks for a total of six doses. Patients were given the same treatment during each subsequent relapse. The main outcome measures were the number of relapses, visual outcome, and ocular complications. RESULTS: The mean follow-up period was 36 months in Group 1 and 30 months in Group 2. The mean number of relapses was significantly reduced and the duration of remission was longer in the infliximab therapy group compared to conventional therapy group (P < .0001). The visual acuity at 24 months follow-up was significantly better in patients treated with infliximab (Group 2) when compared to conventional therapy (Group 1) (P = .0059). CONCLUSIONS: Patients with Behçet disease had significant decrease in inflammation, improvement of visual acuity, and reduced ocular complications following infliximab when compared to conventional therapy. The number of relapses was less in the infliximab treatment group than the conventional therapy group.

Ophthalmic manifestations of Sanjad-Sakati syndrome.

BACKGROUND: Sanjad-Sakati syndrome (SSS) is a rare disorder characterized by hypoparathyroidism, growth and developmental delay, and dysmorphism. The purpose of this report is to describe the ophthalmic manifestations of Sanjad-Sakati syndrome (SSS; hypoparathyroidism-mental retardation-dysmorphism syndrome, HRD) (OMIM 241410). PATIENTS: We included a total of 17 patients who were seen at two hospitals in Riyadh. METHODS: Each patient underwent a complete ophthalmologic evaluation including visual acuity assessment, orthoptic workup, slit-lamp biomicroscopy, intraocular pressure measurement, cycloplegic retinoscopy, funduscopy, corneal diameter, and axial length measurement. RESULTS: All 17 (100%) of the patients had normal visual acuity. All patients had microphthalmia with normal intraocular pressure. Eight (47%) of the patients had esotropia and four (23%) had exotropia. Ophthalmoscopy revealed tortuous retinal blood vessels in all patients. Hyperopic astigmatism was present in 16 (94%) patients. CONCLUSION: Patients with SSS display a variety of ocular findings including errors of refraction, strabismus, and retinal vascular tortuousity.

Ocular manifestations of systemic lupus erythematosus in children.

OBJECTIVE: To determine the prevalence and spectrum of ocular manifestations in children with systemic lupus erythematosus (SLE) and to examine the correlation of the ocular manifestations with disease activity, other organ involvement and the presence of circulating autoantibodies. METHODS: In this cross-sectional study, we performed at King Faisal Specialist Hospital and Research Centre, Riyadh, Kingdom of Saudi Arabia from June 2000 to November 2002, a comprehensive evaluation including detailed eye examination, measuring circulating autoantibodies (antinuclear, antiphospholipid antibodies) and calculation of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). RESULTS: Fifty-two consecutive children (45 females) with SLE completed the evaluation. The mean age of the patients was 11.3 years and the mean SLEDAI was 9.5. Thirty patients (57.7%) had the disease for more than one year. Eighteen patients (34.6%) had ocular manifestations. Seven patients had abnormal Schirmer s test (2 bilateral, 5 unilateral). Five patients had (4 unilateral, one bilateral) retinal vascular lesions. One patient had bilateral iridocyclitis. Three patients had unilateral optic neuropathy and 11 patients had visual field defects (4 bilateral, 7 unilateral). Fisher exact test revealed positive correlation between optic neuropathy and central nervous system (CNS) involvement (p<0.002). There was no correlation among other variables; probably due to the sample size. CONCLUSION: Ocular manifestations including sight threatening complications are not rare in children with SLE. Optic neuropathy has strong prediction for CNS lupus.