Abstract
Purpose :
The objective of this study is to report the clinical, radiological and histopathological characteristics and demographics in Mexican population.
Methods :
Retrospective study, consecutive collation of adults (age range: 33-75 years) who were diagnosed in the Orbit and pathology department of Asocación para Evitar la Ceguera en México, between October 2010- October 2016, with a follow up of 6 months minimum, recording demographics, clinical findings, histopathological diagnosis, orbital and extraorbital involvement, treatment and treatment response.
Results :
10 patients (14 eyes). The mean age was 49.75 years with a SD 12.67 years. There were 60% male and 40% female subjects. 50% had bilateral involvement. 80% lacrimal gland involvement and 20% as an orbital mass. 2 patients (20%) had extraorbital involvement. Six distinct presenting signs of IgG4-ROD were identified, the most common of which were eyelid swelling 100%, proptosis 80%, pain 50%, red eye 20%, diplopia 20% and blurred vision in 10%. The most frequent eye involved was the left eye 70%. The histopathological diagnosis was in 70% dacrioadenitis, 10% follicular lymphoid hiperplasia, 10% Miculikz disease and 10% as an eosinophilic angiocentric fibrosis. The average time between the onset of the symptoms and admision was 2 months to 20 years, with a mean time of 45.8 months (3.8 years). 100% was treated with systemic steroids, 10% with Metrotrexate and 10% with excisional biopsy. 60% had a recovery with systemic streoids.
Conclusions :
IgG4-ROD is an immunological disease, part of a systemic component. Is often misdiagnosed as another entity or underdiagnosed. Is mandatory complete clinical history and physical examination. Steroid is the first line of treatment, new studies shows promising results with Anti-CD20 monoclonal antibodies (Rituximab) as alternative treatment.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.