(CR42) Bilateral Severe Optic Neuritis And Dry Eyes As Initial Presentation of Undifferentiated Connective Tissue Disease; A Rare Occurrence In A Pediatric Patient

Introduction: Undifferentiated Connective Tissue Disease (UCTD) is a rare autoimmune disease that does not meetcriteria for established connective tissue illnesses. Optic nerve involvement in cases of connective tissue disease is rare but optic neuritis can be initial manifestation. We present a case of severe optic neuritis and dry eyes as the initial manifestations of UCTD in a 10-year-old boy. Case Presentation : A 10-year-old Chinese boy with underlying eczema was treated for dry eyes by private ophthalmologist. However, he had gradual bilateral vision worsening for 2 weeks preceded by low-grade fever. Right vision was hand movement while left was counting fingers. Anterior segment showed marked corneal superficial punctate keratitis bilaterally. Fundus showed bilateral optic disc swelling with hyperemia with no evidence of vitritis or retinal vasculitis. Visual evoked potential suggestive of bilateral visual pathway defect. No occult retinal vasculitis on fundus angiogram. Lumbar puncture revealed high opening pressure of 41cmH20 with normal CSF analysis. Connective tissue screening revealed positive autoantibodies. A diagnosis of bilateral severe optic neuritis with dry eyes secondary to UCTD was made. Patient was given IV Methylprednisolone and topical immunomodulators. Dry eyes resolved but vision remained status quo. Cycles of intravenous Cyclophosphamide followed by low dose corticosteroids was commenced. During latest review, visual acuity improved to 1/60 bilaterally despite fundus appearance of bilateral optic atrophy. Discussion/Conclusion : Bilateral severe optic neuritis due to UCTD is a blinding condition. Limited data available regarding therapy. Earlier initiation of second line intensive immunotherapy may lead to better visual prognosis.