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Resumen: Objetivo: reporte de caso de enfermedad relacionada con IgG4 tratada exitosamente con azatioprina. Caso clínico: varón de 53 años con proptosis, cefalea y masa extraocular derecha visible por tomografía, el estudio anatomopatológico concluyó en enfermedad relacionada con IgG4. Se inició tratamiento con prednisona en dosis altas con descenso progresivo, añadiendo azatioprina a los 3 meses para inducción rápida de remisión. Resultados: se observó una mejoría significativa a los 6 meses de tratamiento mixto con prednisona y azatioprina, observándose ausencia de masa extraocular derecha en la resonancia magnética cerebral. Conclusiones: se puede optar por terapia doble con prednisona y azatioprina en un paciente con masa orbitaria debido a enfermedad relacionada con IgG4, ello con la intención de remisión rápida de la enfermedad. Abstract: Objective: case report of IgG4-related disease successfully treated with azathioprine. Clinical case: 53-year-old male with proptosis, headache and right extraocular mass visible by tomography, the pathological study concluded in IgG4-related disease. Treatment with high-dose prednisone was started with progressive reduction, adding azathioprine at 3 months for rapid induction of remission. Results: significant improvement was observed after 6 months of mixed treatment with prednisone and azathioprine, observing the absence of a right extraocular mass in the magnetic resonance imaging brain. Conclusions: dual therapy with prednisone and azathioprine can be chosen in a patient with an orbital mass due to IgG4-related disease, with the intention of rapid remission of the disease.

IgG4 related disease (igG4-RD) is a pathological process which integrates a large number of diseases of unknown pathogenesis, considered as being exclusive to many different organs. Diagnosis is established through histological, radiological and serological criteria. Treatment is based on long term corticosteroids; rituximab being used only in refractory cases. It is unusual for this entity to be found exclusively in the head and neck, without systemic involvement; there are only a few reported cases to date. We present a case of a nasosinusal IgG4-RD orbital tumor with paranasal sinus involvement. The accurate diagnosis made early onset corticosteroid treatment possible and the patient is currently asymptomatic., (Copyright © 2018 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)