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
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