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Article Dans Une Revue American Journal of Ophthalmology Année : 2018

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

L Sarens Inge
  • Fonction : Auteur
Ingele Casteels
  • Fonction : Auteur
Jordi Anton
  • Fonction : Auteur
Brigitte Bader-Meunier
Philippe Brissaud
  • Fonction : Auteur
Gaelle Chedeville
  • Fonction : Auteur
Rolando Cimaz
  • Fonction : Auteur
D Dick Andrew
  • Fonction : Auteur
Graciella Espada
  • Fonction : Auteur
Jorge Fernandez-Martin
  • Fonction : Auteur
M Guly Catherine
  • Fonction : Auteur
Eric Hachulla
Miroslav Harjacek
  • Fonction : Auteur
Raju Khubchandani
  • Fonction : Auteur
Friederike Mackensen
  • Fonction : Auteur
Rosa Merino
  • Fonction : Auteur
Consuelo Modesto
  • Fonction : Auteur
Antonio Naranjo
  • Fonction : Auteur
Sheila Oliveira-Knupp
  • Fonction : Auteur
Seza Ozen
  • Fonction : Auteur
Christine Pajot
  • Fonction : Auteur
V Ramanan Athimalaipet
  • Fonction : Auteur
Ricardo Russo
  • Fonction : Auteur
Gordana Susic
  • Fonction : Auteur
Akaluck Thatayatikom
  • Fonction : Auteur
Caroline Thomee
  • Fonction : Auteur
Sebastiaan Vastert
  • Fonction : Auteur
John Bertin
  • Fonction : Auteur
I Arostegui Juan
  • Fonction : Auteur
D Rose Carlos
  • Fonction : Auteur
H Wouters Carine
  • Fonction : Auteur

Résumé

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.

Dates et versions

hal-04318007 , version 1 (01-12-2023)

Identifiants

Citer

L Sarens Inge, Ingele Casteels, Jordi Anton, Brigitte Bader-Meunier, Philippe Brissaud, et al.. Blau Syndrome-Associated Uveitis: Preliminary Results From an International Prospective Interventional Case Series. American Journal of Ophthalmology, 2018, American Journal of Ophthalmology, 187, pp.158-166. ⟨10.1016/j.ajo.2017.08.017⟩. ⟨hal-04318007⟩

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