Please use this identifier to cite or link to this item: http://hdl.handle.net/10553/50090
Title: Blau Syndrome–Associated Uveitis: preliminary results from an international prospective interventional case series
Authors: Sarens, Inge L.
Casteels, Ingele
Anton, Jordi
Bader-Meunier, Brigitte
Brissaud, Philippe
Chédeville, Gaelle
Cimaz, Rolando
Dick, Andrew D.
Espada, Graciella
Fernandez-Martin, Jorge
Guly, Catherine M.
Hachulla, Eric
Harjacek, Miroslav
Khubchandani, Raju
Mackensen, Friederike
Merino, Rosa
Modesto, Consuelo
Naranjo, Antonio 
Oliveira-Knupp, Sheila
Özen, Seza
Pajot, Christine
Ramanan, Athimalaipet V.
Russo, Ricardo
Susic, Gordana
Thatayatikom, Akaluck
Thomée, Caroline
Vastert, Sebastiaan
Bertin, John
Arostegui, Juan I.
Rose, Carlos D.
Wouters, Carine H.
UNESCO Clasification: 320109 Oftalmología
Issue Date: 2018
Journal: American journal of ophthalmology 
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.
URI: http://hdl.handle.net/10553/50090
ISSN: 0002-9394
DOI: 10.1016/j.ajo.2017.08.017
Source: American Journal of Ophthalmology [ISSN 0002-9394], v. 187, p. 158-166
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