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María de los Angele Ramos Cadena, Gustavo Aguilar Montes, Matilde Ruiz Cruz; Posterior Scleritis and Orbital Mass Associated to Positive Antineutrophil Cytoplasmic Autoantibodies Without Systemic Involvement. Invest. Ophthalmol. Vis. Sci. 2012;53(14):6250.
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To present a clinical case of a 16 year-old Mexican patient, without any relevant previous medical history and systemic symptoms, diagnosed with posterior scleritis associated to positive antineutrophil cytoplasmic autoantibodies (ANCA) directed to Proteinase 3 (PR3), with clinical involvement of the right eye.
We performed a complete ophthalmologic examination, laboratory and immunologic tests, ocular ultrasound, orbit and paranasal sinus CT Scan and cranial MRI.
Funduscopic examination revealed papillitis, macular scar, serous retinal detachment, and vitritis. Ocular ultrasound of the right eye showed T-sign, enlargement of the posterior wall and intraorbital optic nerve. Laboratory results did not show renal or other systemic involvement. ANCA results showed positive cANCA, and Anti-PR3. CT Scan revealed a small mass in the orbit cone, close to the optic nerve entrance, and enlargement of the intraorbital portion of the optic nerve. MRI disclosed a normal intracraneal optic nerve.She was treated with intravenous methylprednisolone 1g per day, during three days, followed by oral prednisone at immunosuppressive dose, tapered according to evolution, discontinued after one month and a half. The ophthalmologic and imagenologic manifestations ceased after the first month of treatment.
50% of posterior scleritis are related to systemic involvement. This patient presented positive cANCA and Anti-PR3 even after corticosteroid treatment, which would usually imply systemic activity; but in this case the disease presented without extraocular manifestations. Immunosuppressive treatment should be indicated if systemic involvement occurs or ocular symptoms recur because of the high risk of vasculitic multiorgan affection and renal failure.
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