IgG4-related ophthalmic disease

IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease,[2] which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures.

[2][3] It basically can cause loss of vision and in other cases, cause skin irritation and loss of brain function Symptoms, if any, can be mild even in the presence of significant swelling or masses.

Other orbital masses or inflammation can result in visual disturbance (blurred vision, double vision, visual field impairment), restricted eye movements, pain or discomfort, numbness in the distribution of the supraorbital and/or infraorbital nerves, or proptosis.

[1][3] The extent of inflammation that can occur in IgG4-ROD is well demonstrated on magnetic resonance imaging (MRI).

[citation needed] Infraorbital nerve enlargement (IONE) is considered to be a particularly suspicious sign of IgG4-ROD, but seems to occur only when inflammation is in direct contact with the infraorbital canal.

Breakdown of diagnosis in orbital lymphoproliferative disorders in a Japanese study [ 1 ]
Enlargements in the left inferior rectus muscle and infraorbital nerve (arrow) in a 65-year-old man with a serum IgG4 of 404 mg/dL. [ 1 ] (T2-weighted MRI)
Swelling of the left superior and lateral rectus muscles , a mass lesion around the left optic disc (arrow) , and enlargements of the left supraorbital nerve and the right infraorbital nerve (arrow heads) in a 60-year-old man with a serum IgG4 of 463 mg/dL. [ 1 ] (T1-weighted MRI)
Bilateral supraorbital nerve enlargements (arrows) and right infraorbital nerve (arrow head) enlargement in a 47-year-old woman with a serum IgG4 of 1000 mg/dL. [ 1 ] (T1-weighted MRI)