Blau Syndrome-Associated Uveitis: Preliminary ...
Type de document :
Article dans une revue scientifique
PMID :
URL permanente :
Titre :
Blau Syndrome-Associated Uveitis: Preliminary Results From an International Prospective Interventional Case Series
Auteur(s) :
Sarens Inge, L [Auteur]
Casteels, Ingele [Auteur]
Anton, Jordi [Auteur]
Bader-Meunier, Brigitte [Auteur]
Brissaud, Philippe [Auteur]
Chedeville, Gaelle [Auteur]
Cimaz, Rolando [Auteur]
Dick Andrew, D [Auteur]
Espada, Graciella [Auteur]
Fernandez-Martin, Jorge [Auteur]
Guly Catherine, M [Auteur]
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Harjacek, Miroslav [Auteur]
Khubchandani, Raju [Auteur]
Mackensen, Friederike [Auteur]
Merino, Rosa [Auteur]
Modesto, Consuelo [Auteur]
Naranjo, Antonio [Auteur]
Oliveira-Knupp, Sheila [Auteur]
Ozen, Seza [Auteur]
Pajot, Christine [Auteur]
Ramanan Athimalaipet, V [Auteur]
Russo, Ricardo [Auteur]
Susic, Gordana [Auteur]
Thatayatikom, Akaluck [Auteur]
Thomee, Caroline [Auteur]
Vastert, Sebastiaan [Auteur]
Bertin, John [Auteur]
Arostegui Juan, I [Auteur]
Rose Carlos, D [Auteur]
Wouters Carine, H [Auteur]
Casteels, Ingele [Auteur]
Anton, Jordi [Auteur]
Bader-Meunier, Brigitte [Auteur]
Brissaud, Philippe [Auteur]
Chedeville, Gaelle [Auteur]
Cimaz, Rolando [Auteur]
Dick Andrew, D [Auteur]
Espada, Graciella [Auteur]
Fernandez-Martin, Jorge [Auteur]
Guly Catherine, M [Auteur]
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Harjacek, Miroslav [Auteur]
Khubchandani, Raju [Auteur]
Mackensen, Friederike [Auteur]
Merino, Rosa [Auteur]
Modesto, Consuelo [Auteur]
Naranjo, Antonio [Auteur]
Oliveira-Knupp, Sheila [Auteur]
Ozen, Seza [Auteur]
Pajot, Christine [Auteur]
Ramanan Athimalaipet, V [Auteur]
Russo, Ricardo [Auteur]
Susic, Gordana [Auteur]
Thatayatikom, Akaluck [Auteur]
Thomee, Caroline [Auteur]
Vastert, Sebastiaan [Auteur]
Bertin, John [Auteur]
Arostegui Juan, I [Auteur]
Rose Carlos, D [Auteur]
Wouters Carine, H [Auteur]
Titre de la revue :
American Journal of Ophthalmology
Nom court de la revue :
Am. J. Ophthalmol.
Numéro :
187
Pagination :
158-166
Date de publication :
2018-03-01
ISSN :
0002-9394
Discipline(s) HAL :
Sciences du Vivant [q-bio]
Résumé en anglais : [en]
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 ...
Lire la suite >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.Lire moins >
Lire la suite >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.Lire moins >
Langue :
Anglais
Audience :
Internationale
Vulgarisation :
Non
Établissement(s) :
Inserm
Université de Lille
CHU Lille
Université de Lille
CHU Lille
Équipe(s) de recherche :
Immunity, inflammation and fibrsis in auto and allo-reactivity
Date de dépôt :
2019-03-01T14:17:28Z
2023-12-01T13:10:22Z
2023-12-01T13:10:22Z