A Diagnostic Potpourri (video)

The presence of sclerosing fibro-proliferative orbital pathology involving both orbits raised the suspicion that it was associated with a systemic inflammation since patient was known to have RA, cutaneous lupus and Hepatitis C. An extensive autoimmune work-up revealed elevated c-ANCA (1:20 titer- not known previously), negative p-ANCA staining, negative anti-protease 3 and anti-myeloperoxidase and negative ANA. He also had elevated anti-CCP 91 units/mL, and rheumatoid factor 110 IU/mL . This profile suggested a new diagnosis of an atypical c-ANCA positive autoimmune vasculitis such as atypical granulomatosis polyangitis. We believe this resulted in orbital inflammation with occlusive vasculitis leading to ophthalmic artery occlusion and ocular ischemia. Repeat MRI orbit continued to show abnormal enhancing soft tissue signal within bilateral orbits. He was treated with 60 mg prednisone mg and started on rituximab infusions subsequently. A month later he developed neovascular glaucoma for which he underwent pan retinal laser photocoagulation and placed on intraocular pressure lowering eye drops. His right eye remains asymptomatic with a visual acuity of 20/30 and left eye has no light perception. His history of hepatitis C and his incarcerated status led to administrative delays in initiating Rituximab for the current ANCA positive vasculitis. Low dose prednisone failed to control the orbital inflammation adequately and led to occlusive vasculitis causing his left eye vision loss.