P-ANCA Vasculitis Presenting with Orbital Infiltration

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) may affect the eye or orbit, and ophthalmic manifestations of AAV are associated with higher mortality than other inflammatory eye diseases. Perinuclear ANCA (p-ANCA) vasculitis is an uncommon cause of orbital inflammation. A 70-year-old woman with chronic kidney disease presented with a 1-year history of orbital mass and edema around her OD. Fundoscopy revealed 360° optic disc elevation OD. MRI orbits showed an infiltrative, intra- and extraconal lesion extending through the right orbital apex to the cavernous sinus. Labwork and orbital biopsy were consistent with p-ANCA vasculitis, and the patient's ocular symptoms improved after methylprednisolone. Diagnosis of AAV is complicated by a wide diversity of symptoms, and this case highlights an unusual presentation of p-ANCA vasculitis in the orbit. Ophthalmologists have an important role in diagnosing systemic conditions such as AAV by initiating the proper inflammatory workup.