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Blau Syndrome-Associated Uveitis: Preliminary Results From an International Prospective Interventional Case Series

Sarens, IL; Casteels, I; Anton, J; Bader-Meunier, B; Brissaud, P; Chédeville, G; Cimaz, R; ... Wouters, CH; + view all (2018) Blau Syndrome-Associated Uveitis: Preliminary Results From an International Prospective Interventional Case Series. American Journal of Ophthalmology , 187 pp. 158-166. 10.1016/j.ajo.2017.08.017. Green open access

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Abstract

PURPOSE: Provide baseline and preliminary follow-up results in a 5-year longitudinal study of Blau syndrome. DESIGN: Multicenter, prospective interventional case series. METHODS: Baseline data from 50 patients from 25 centers worldwide, and follow-up data for patients followed 1, 2, or 3 years at the end of study enrollment. Ophthalmic data were collected at baseline and yearly visits by means of a standardized collection form. RESULTS: Median age at onset of eye disease was 60 months and duration of eye disease at baseline 145 months. At baseline 38 patients (78%) had uveitis, which was bilateral in 37 (97%). Eight patients (21%) had moderate to severe visual impairment. Panuveitis was found in 38 eyes (51%), with characteristic multifocal choroidal infiltrates in 29 eyes (39%). Optic disc pallor in 9 eyes (12%) and peripapillary nodules in 9 eyes (12%) were the commonest signs of optic nerve involvement. Active anterior chamber inflammation was noted in 30 eyes (40%) at baseline and in 16 (34%), 17 (57%), and 11 (61%) eyes at 1, 2, and 3 years, respectively. Panuveitis was associated with longer disease duration. At baseline, 56 eyes (75%) were on topical corticosteroids. Twenty-six patients (68%) received a combination of systemic corticosteroids and immunomodulatory therapy. CONCLUSIONS: Blau uveitis is characterized by progressive panuveitis with multifocal choroiditis, resulting in severe ocular morbidity despite continuous systemic and local immunomodulatory therapy. The frequency and severity of Blau uveitis highlight the need for close ophthalmologic surveillance as well as a search for more effective therapies.

Type: Article
Title: Blau Syndrome-Associated Uveitis: Preliminary Results From an International Prospective Interventional Case Series
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ajo.2017.08.017
Publisher version: https://doi.org/10.1016/j.ajo.2017.08.017
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Adolescent, Adult, Antihypertensive Agents, Arthritis, Child, Child, Preschool, Choroiditis, Cross-Sectional Studies, Disease Progression, Female, Follow-Up Studies, Global Health, Glucocorticoids, Humans, Immunosuppressive Agents, Infant, Male, Middle Aged, Prospective Studies, Synovitis, Uveitis, Visual Acuity
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10074213
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