Can IgG4 affect the eyes?
Patients with IgG4-ROD are at increased risk for lymphoma, particularly low-grade extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Untreated IgG4-ROD affecting the lacrimal glands may also lead to irreversible damage and lifelong dry eye disease.
Is IgG4 disease serious?
IgG4-related disease has been described as an indolent condition. Although possibly based on opinion rather than on objective assessments, symptoms, if any, are commonly described as mild in the medical literature. This can be in spite of considerable underlying organ destruction.
Can IgG4 affect the brain?
Importance IgG4-related disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells. Head and brain involvement is rare in IgG4-RD, and brain parenchyma involvement has never been reported.
What is IgG4 eye disease?
IgG4-related ophthalmic disease (IgG4-ROD) is the recommended term to describe orbital (eye socket) manifestations of the systemic condition IgG4-related disease, which is characterised by infiltration of lymphocytes and plasma cells and subsequent fibrosis in involved structures.
Can IgG4 be cured?
It may also lead to swelling that can be mistaken for tumors. Particularly if diagnosed early, IgG4-RSD is highly treatable.
How is IgG4 treated?
IgG4-related disease is usually treated with systemic steroids, often prednisone 40 mg per day for 2–4 weeks followed by a gradual tapering of the dose. In patients that cannot be taken off prednisone, a steroid-sparing agent like azathioprine or mycophenolate may be used.
What kind of disease is IgG4 related orbital inflammation?
IgG4-related disease (IgG4-RD) is a systemic condition in which fibroinflammatory lesions rich in IgG4+ plasma cells can be present in single or multiple organs of the body. When the disease manifests in the eye or orbit, it is referred to as IgG4-related ophthalmic disease (IgG4-ROD),…
What’s the difference between IgG 4 rod and IgG4 rod?
IgG4-RD is a systemic inflammatory disorder in which fibrosing lesions may be found in various areas and organs of the body. IgG4-RD is the umbrella term for systemic disease, while IgG4-ROD is the more specific term for manifestations in the eye and orbit.
When was IgG4-RD first presented to the public?
IgG4-RD was first presented in 2001 in a Japanese study of patients with autoimmune pancreatitis (AIP) who were found to have elevated serum IgG4 levels.
How are lacrimal glands affected by IgG4-ROD?
Lacrimal glands affected by IgG4-ROD have previously been found to demonstrate somatic hypermutation, suggesting local immune response to antigen.