Escleritis necrotizante c-ANCA positivo y esclerosis múltiple compatible con Wegener ocular: tratamiento con rituximab / C-ANCA positive necrotising scleritis and multiple sclerosis compatible with ocular Wegener: treatment with rituximab

CASO CLÍNICO: Paciente diagnosticada de escleritis necrotizante c-ANCA+, seudotumor orbitario y posible esclerosis múltiple; en 2003 realizó tratamiento con ciclofosfamida oral y esteroides con respuesta parcial. Entre 2005-2010 sufrió episodios oculares autolimitados. En 2010 se realizó un primer trasplante escleral, con mala evolución. Se inició tratamiento con rituximab y se realizó segundo injerto con buena evolución. A los 12 meses no se observaron cambios en resonancia magnética y el segundo injerto cicatrizó. DISCUSIÓN: La enfermedad de Wegener con afectación limitada a la órbita y/o el ojo es una entidad poco frecuente. La anatomía patológica, analítica de sangre, clínica y buena respuesta al tratamiento son clave para su diagnóstico

CASE REPORT: A patient diagnosed with necrotizing scleritis, c-ANCA + an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005-2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed. DISCUSSION: Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis

[C-ANCA positive necrotising scleritis and multiple sclerosis compatible with ocular Wegener: treatment with rituximab]. / Escleritis necrotizante c-ANCA positivo y esclerosis múltiple compatible con Wegener ocular: tratamiento con rituximab.

CASE REPORT: A patient diagnosed with necrotizing scleritis, c-ANCA+ an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005-2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed. DISCUSSION: Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis.