Intravenous tocilizumab has been demonstrated to be an effective disease modifying agent in the treatment of moderate to severe active thyroid eye disease. The authors describe 2 patients treated with tocilizumab using home subcutaneous administration over an accelerated, biweekly course. Both patients exhibited improvement in thyroid eye disease clinical activity and proptosis.
External beam radiation therapy for localized periocular AL amyloidosis demonstrated efficacy at halting disease progression at one year; however, the long-term efficacy is unknown. Monitoring of periocular amyloid is best achieved with slit lamp exam and external photography as opposed to MRI.
A 68-year-old man with known history of IgG4-related disease presented with a 1-year history of mucoid discharge OD and progressive proptosis OS. MRI showed diffuse enlargement of the left lacrimal gland along with prolonged infiltrative changes involving the left extraocular muscles, supraorbital nerve, and infraorbital nerve. No identifiable masses were noted in either nasolacrimal drainage system on MRI. Due to clinical evidence of nasolacrimal duct obstruction, a right external dacryocystorhinostomy was performed along with biopsies of the right lacrimal sac and left lacrimal gland. Immunohistochemical stains identified IgG4-positive plasma cells in the biopsy of the right lacrimal sac indicative of secondary acquired nasolacrimal duct obstruction despite the absence of mass forming lesions on exam and nasolacrimal imaging. This report is the first to describe nasolacrimal drainage system IgG4-related ophthalmic disease involvement in an otherwise clinically and radiographically normal appearing tear drainage system.
A 14-year-old female with juvenile idiopathic arthritis presented with left-sided recurrent painful periorbital swelling of 5 months duration. MRI revealed enlargement of the left lacrimal gland with extensive inflammatory change involving the orbital and periorbital soft tissues. Lacrimal gland biopsy showed evidence of chronic inflammation, and she had intralesional steroid injection at the time of biopsy. She remained asymptomatic at 6-month follow-up visit. Although juvenile idiopathic arthritis is usually related to intraocular inflammation, it may rarely be associated with dacryoadenitis.
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