Papillitis with retinal venous congestion and intraocular inflammation

PURPOSE: To describe a case of pronounced papillitis with diminished venous outflow and vitreous inflammation in a 50-year-old man who was later found to have clinical and serological manifestations of dermatomyositis. OBSERVATIONS: A 50-year-old man presented with papillitis associated with venous congestion and intraocular inflammation. He was normotensive, not on medications, and without known heritable hypercoagulable or inflammatory disease. Review of systems revealed axial and proximal muscle pain involving the lower back, hip, and thigh, and he developed a transient rash of the scalp. His evaluation including infectious, hypercoagulable, and inflammatory etiologies was negative except for a significantly elevated Mi-2 antibody titer. Treatment with intravenous and oral steroids improved the papillitis, visual acuity and visual field deficit. CONCLUSIONS: Significant papillitis and retinal venous stasis with intraocular inflammation may be associated with dermatomyositis. IMPORTANCE: Rapidly progressive optic disc edema with associated inflammation and venous stasis requires a broad work up for infectious, hypercoagulable, and autoimmune etiologies for targeted therapy and visual preservation. To the best of our knowledge, this may be the first reported clinical presentation of dermatomyositis manifesting initially with papillitis and retinal venous congestion, based upon elevated Mi-2 antibodies and additional clinical features.