Blau-Jabs Syndrome in a Tertiary Ophthalmologic Center

Blau-Jabs Syndrome in a Tertiary Ophthalmologic Center

Author Nascimento, Heloisa Autor UNIFESP Google Scholar
Sousa, Jacqueline M. Autor UNIFESP Google Scholar
Fernandez, Delia Gonzalez Autor UNIFESP Google Scholar
Salomao, Gustavo H. A. Autor UNIFESP Google Scholar
Sato, Elcio H. Autor UNIFESP Google Scholar
Muccioli, Cristina Autor UNIFESP Google Scholar
Belfort, Rubens, Jr. Autor UNIFESP Google Scholar
Abstract In a prospective case series of patients with Blau-Jabs syndrome (BJS) conducted in the Ophthalmology Department/Federal University of Sao Paulo, seven patients math clinical and ophthalmologic manifestations of the disease and a positive genetic test result for the presence of a mutation in the CARD15/NOD2 gene were followed for a minimal period of 1 year. All patients had uveitis, five had nummular corneal subepithelial opacities, and four had multifocal choroiditis. Oral prednisolone was administered to all patients

inflammation was controlled in six patients with at least one immunosuppressive drug. Infliximab (Remicade

Janssen Pharmaceuticals, Beerse, Belgium) and etanercept (Enbrel

Amgen, Thousand Oaks, CA) were used to treat two cases refractory to the antiinflammatory drugs. A subconjunctival dexamethasone implant (Ozurdex

Allergan, Irvine, CA) and a periocular injection of triamcinolone were used in one case to achieve inflammation control. Six patients achieved a visual acuity of 20/25 or better. The authors conclude that periocular treatment with steroid injections might be effective adjuvant therapy to control ocular inflammation.
xmlui.dri2xhtml.METS-1.0.item-coverage Thorofare
Language English
Date 2018
Published in Ophthalmic Surgery Lasers & Imaging Retina. Thorofare, v. 49, n. 1, p. 70-75, 2018.
ISSN 2325-8160 (Sherpa/Romeo, impact factor)
Publisher Slack Inc
Extent 70-75
Origin http://dx.doi.org/10.3928/23258160-20171215-12
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000419990200012
URI https://repositorio.unifesp.br/handle/11600/53925

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