Blau-Jabs Syndrome in a Tertiary Ophthalmologic Center
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Data
2018
Autores
Nascimento, Heloisa 
Sousa, Jacqueline M.
Fernandez, Delia Gonzalez
Salomao, Gustavo H. A.
Sato, Elcio H.
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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.
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.
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Ophthalmic Surgery Lasers & Imaging Retina. Thorofare, v. 49, n. 1, p. 70-75, 2018.