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Start free with EleplanIgG4-related ophthalmic disease
ORPHA:449563Clinical subtype
What it is
A rare, inflammatory eye disease characterized by IgG4-immunopositive lymphocyte and plasmacyte infiltration and collagenous fibrosis of affected tissue and elevated serum levels of IgG4. Clinical presentation includes mass lesion or swelling of the involved structures, commonly involving lacrimal gland and duct, infraorbital and supraorbital nerves, extraocular muscles and orbital soft tissues. A systemic involvement is common.
Key facts
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Clinical subtype
Signs and symptoms
Common30–79%
16- Abnormal fifth cranial nerve morphology
- Abnormality of infra-orbital nerve
- Abnormality of the orbital region
- Abnormal lacrimal gland morphology
- Allergy
- Antinuclear antibody positivity
- Dacryocystitis
- Elevated circulating C-reactive protein concentration
- Enlarged lacrimal glands
- Increased circulating IgE concentration
- Increased circulating IgG4 level
- Increased total eosinophil count
- Lymphadenopathy
- Pancreatitis
- Periorbital edema
- Sialadenitis
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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