| Presentation preference | Oral presentation |
| Title | IgG4-Related Disease Isolated to the Orbit |
| Accept poster if oral is not possible ? | Yes |
| Purpose | To report on a series of IgG4-related disease isolated to the ophthalmic orbit, with manifestations that include myopathy and neuropathy. The isolated involvement of the orbit has mechanistic implications regarding the pathogenesis of disease. |
| Methods | Multi-institutional retrospective chart review was performed, identifying 9 patients with isolated orbital IgG4-related disease. Clinical, histologic and radiologic findings were analyzed, along with response to treatment. |
| Results | Patients were primarily male, middle aged, manifesting with severe myopathy, with frequent trigeminal nerve involvement. Biopsy was performed in 8 of the 9 cases. Definitive diagnosis of IgG4-related disease was made in 6 of 9 cases, and probable in the other 3 cases. All patients were treated with either systemic or orbital corticosteroids, and some with both. Patient response to CD20-inhibition (rituximab) was documented. |
| Conclusion | IgG4 is an IgG subtype that serves as the end-product of a Th2/Treg cytokine-driven response to immune system activation. When the immune system activation is systemic, the Th2/Treg response is expected to be systemic. However, when the inflammation is end-organ restricted, the Th2/Treg response is also restricted, leading to isolated end-organ involvement with IgG4 deposition. We propose that IgG4-related disease is better termed “Th2/Treg Modulated Disease,” reflecting the pathophysiology of the disease rather than the post-facto histologic and laboratory findings. |
| Conflict of interest | No |
1
| Last name | ZWETKOFF |
| Initials of first name(s) | A |
| Department | Hospital das Clínicas – UFMG |
| City | Belo Horizonte, MG |
| Country | Brazil |
2
| Last name | Mingardo |
| Initials of first name(s) | F |
| Department | Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan |
| City | Ann Arbor, MI |
| Country | United States |
3
| Last name | Kahana |
| Initials of first name(s) | A |
| Department | Kahana Oculoplastic and Orbital Surgery |
| City | Livonia, MI |
| Country | United States |
4
| Last name | Elner |
| Initials of first name(s) | V M |
| Department | Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan |
| City | Ann Arbor, MI |
| Country | United States |
