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Katarzyna Powierza,1 Jolanta Sawicka-Powierza,2 Beata Urban,3 Beata Żelazowska-Rutkowska,4 Bogdan Cylwik,4 Bożena Mikołuć,5 Olga Kowalewska,6 Alina Bakunowicz-Łazarczyk3 1University Clinical Hospital in Bialystok, Medical University of Bialystok, Bialystok 15-276, Poland; 2Department of Family Medicine, Medical University of Bialystok, Bialystok 15-054, Poland; 3Department of Pediatric Ophthalmology and Strabismus, Medical University of Bialystok, Bialystok 15-274, Poland; 4Department of Pediatric Laboratory Diagnostics, Medical University of Bialystok, Bialystok 15-274, Poland; 5Department of Pediatrics, Rheumatology, Immunology and Metabolic Bone Diseases, Medical University of Bialystok, Bialystok 15-274, Poland; 6Psychiatric Clinic, Lund, Division of Psychiatry, Lund 222 40, SwedenCorrespondence: Jolanta Sawicka-PowierzaDepartment of Family Medicine, Medical University of Bialystok, Mieszka I 4B Street, Bialystok 15-054, PolandTel +48 857326820Fax +48 857327848Email jolasawicka@gmail.comPurpose: The aim of our study was to determine endothelin-1 (ET-1) concentration in chronic idiopathic uveitis in children and adolescents depending on anatomical location and grade of inflammation.Methods: The cross-sectional study was conducted among 17 patients with chronic idiopathic uveitis and 22 healthy controls. Concentration of ET-1 in serum was determined using a commercially available ELISA kit. The concentration of C reactive protein (CRP) in serum was determined by immunoturbidimetric method using CRP4 reagent kit (Roche Diagnostics GmbH, Mannheim, Germany).Results: Statistically significant difference between ET-1 concentration in patients with chronic idiopathic uveitis and controls was found 1.33 (1.22; 1.48) vs 1.93 (1.1; 3.11), p = 0.008). No correlations were found between ET-1 concentration and age, either in chronic idiopathic uveitis patients or controls. Nine out of 17 patients presented with anterior uveitis, 5 with posterior and 3 with panuveitis. There were no differences in ET-1 concentration between anterior, posterior and panuveitis (p = 0.634), and in terms of grade of inflammation.Conclusion: ET-1 expression is disturbed in pediatric chronic idiopathic uveitis irrespective of the anatomical location and grade of inflammation. Lower expression of ET-1 plays a crucial role in disturbed vascular tone control and can result in permanent visual impairment in chronic non-infectious uveitis.Keywords: adolescents, children, chronic idiopathic uveitis, endothelin-1

Abstract In January 2013, a 54-year-old pseudophakic woman was referred to the ocular inflammation clinic with a diagnosis of panuveitis, already on oral cyclosporine (100 mg/day) since 2010. She was a monocular patient, with the left eye eviscerated due to trauma and secondary endophthalmitis in 1995. She had experienced similar recurring episodes between 2010 and 2013. Examination revealed best-corrected visual acuity (BCVA) of 20/200. The anterior segment revealed the presence of flare and absence of cells. Intraocular pressure (IOP) was 11 mmHg. Fundus examination revealed the presence of anterior vitreous organization, exuberant cystoid macular edema (CME) and peripheral Dalen-Fuchs nodules with no activity. Systemic workup showed no changes, and a presumptive diagnosis of sympathetic ophthalmia was made. Initial therapy included topical and systemic corticosteroids. Cyclosporine was replaced with methotrexate (20 mg/week) due to nephrotoxicity. There was marked improvement in anterior inflammation and vitreous organization, but the ME remained exuberant (central ring thickness of 0.7 mm). Over the subsequent 4 years of follow-up, the patient received two intravitreous injections of a dexamethasone extended-release implant (Ozurdex®), with normalization of macular thickness and improvement in visual acuity (VA), but ME recurred around 6 months post-injection. During this period, the patient also underwent three sub-tenon injections of triamcinolone, with marked improvement of ME, but ME recurred between 4 and 6 months after treatment. She was thus recommended for treatment with a fluocinolone extended-release implant (ILUVIEN®). Post-implantation (3-year follow-up), she had controlled anterior inflammation without ME (central macular thickness of 248 µm). This case suggests that sustained control of inflammation enables better control of ME, and benefits may persist even after cessation of the direct anti-inflammatory effect of the implant. Funding Alimera Sciences Ltd funded the Rapid Service Fees.

In January 2013, a 54-year-old pseudophakic woman was referred to the ocular inflammation clinic with a diagnosis of panuveitis, already on oral cyclosporine (100 mg/day) since 2010. She was a monocular patient, with the left eye eviscerated due to trauma and secondary endophthalmitis in 1995. She had experienced similar recurring episodes between 2010 and 2013. Examination revealed best-corrected visual acuity (BCVA) of 20/200. The anterior segment revealed the presence of flare and absence of cells. Intraocular pressure (IOP) was 11 mmHg. Fundus examination revealed the presence of anterior vitreous organization, exuberant cystoid macular edema (CME) and peripheral Dalen-Fuchs nodules with no activity. Systemic workup showed no changes, and a presumptive diagnosis of sympathetic ophthalmia was made. Initial therapy included topical and systemic corticosteroids. Cyclosporine was replaced with methotrexate (20 mg/week) due to nephrotoxicity. There was marked improvement in anterior inflammation and vitreous organization, but the ME remained exuberant (central ring thickness of 0.7 mm). Over the subsequent 4 years of follow-up, the patient received two intravitreous injections of a dexamethasone extended-release implant (Ozurdex®), with normalization of macular thickness and improvement in visual acuity (VA), but ME recurred around 6 months post-injection. During this period, the patient also underwent three sub-tenon injections of triamcinolone, with marked improvement of ME, but ME recurred between 4 and 6 months after treatment. She was thus recommended for treatment with a fluocinolone extended-release implant (ILUVIEN®). Post-implantation (3-year follow-up), she had controlled anterior inflammation without ME (central macular thickness of 248 µm). This case suggests that sustained control of inflammation enables better control of ME, and benefits may persist even after cessation of the direct anti-inflammatory effect of the implant. Funding: Alimera Sciences Ltd funded the Rapid Service Fees. [ABSTRACT FROM AUTHOR]