Dr.MANDABI SENGUPTA, M21530
IgG4 related disease is a multisystem immune-mediated fibroinflammatory condition, commonly affecting adults and is characterized by elevated serum IgG4 level and IgG4-positive lymphoplasmacytic infiltrations in different organs. Ophthalmic involvement is seen in 4-34% cases. Lacrimal gland is the most common ocular site of involvement. Extraocular muscles, orbital soft tissues, eyelids, orbital bones, sclera and choroid may also be involved.
A 40 year old man presented with swelling of upper eyelid with pain and redness of right eye of 10 days duration. Based on ocular examination scleritis was suspected. Laboratory investigations revealed lymphocytosis, raised CRP, ESR and serum IgG4 level. A provisional diagnosis of IgG4 related ophthalmic disease was made and treatment with systemic corticosteroid showed significant improvement.
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