Purpose/methods: A 72-year-old woman with antineutrophil cytoplasmic antibody-positive vasculitis had a combined detachment of the choroid and retina. This unique initial manifestation was also associated with systemic and orbital manifestations, including brow ptosis, dacryoadenitis, and pneumonitis.
Results/conclusions: This patient underwent vitrectomy for removal of a dense vitreitis and responded well to systemic cyclophosphamide. This case demonstrates that antineutrophil cytoplasmic antibody testing is useful for the diagnosis and treatment of Wegener's granulomatosis and microscopic polyarteritis-associated scleritis. The distinction between these two entities is often difficult to make.