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Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients - AJNR News Digest
November-December 2015
Head & Neck

Immunoglobulin G4–Related Disease of the Orbit: Imaging Features in 27 Patients

Laurence J. Eckel, MD

Laurence J. Eckel, MD

I chose to research this topic because of close collaboration with the ophthalmology department and a desire to provide a better differential diagnosis when interpreting images of abnormal orbital findings on CT and MRI.  Radiographically, we were often not recognizing or suggesting the possibility of IgG4-related disease (IgG4-RD) of the orbit and we wanted to discover how we could get better.

In brief, our research discovered that certain radiographic features were more common in IgG4-RD than in other diseases that can look similar. In particular, IgG4-RD can be suggested when extraocular muscle enlargement is bilateral, usually involving the lateral rectus more than other muscles. In addition, other supporting features such as lacrimal gland involvement, infraorbital nerve involvement, and an infiltrative process in the orbital fat can also be suggestive of IgG4-RD. From this research we developed a decision tree for deciding whether orbital disease is more likely to be IgG4-related when compared to other similarly appearing diseases, such as thyroid ophthalmopathy.

The findings have substantially influenced our practice in that we are able to better guide testing and clinical management. Whereas, before, the diagnosis of IgG4-RD was rarely entertained, it is now better recognized by radiologists and clinicians, enabling more rapid diagnosis and treatment.

The findings have influenced my clinical practice in several ways. First, the collaboration with the clinical service has resulted in a more satisfactory and specialized practice. It has also opened the doors to additional related research. We have also received several requests for additional information, and our images have been used at national meetings by various people speaking about IgG4-RD of the orbit.

Based on our work and collaboration, we have already expanded our research into diseases that look similar, such as thyroid and Wegener granulomatosis and pseudotumor. In addition, we are expanding our efforts in other orbital diseases unrelated to IgG4.

We just presented several abstracts at the American Society of Head and Neck Radiology annual meeting in Naples, Florida and will continue to present as new discoveries and data become available.

 

Read this article at AJNR.org …