P-ANCA Vasculitis Presenting with Orbital Infiltration-Reference-Cited by-同舟云学术

P-ANCA Vasculitis Presenting with Orbital Infiltration

Published:2024-01-17 Issue: Volume: Page:
ISSN:0740-9303
Container-title:Ophthalmic Plastic & Reconstructive Surgery
language:en
Short-container-title:

Author:

Yoo Kristy G.¹,Abella-Ayala Frank¹²,Lerner Alexander¹³,Kwok Alyssa¹⁴,Chang Jessica R.¹²

Affiliation:

1. Keck School of Medicine at the University of Southern California, Los Angeles, California, U.S.A.

2. Department of Ophthalmology, USC Roski Eye Institute, Keck School of Medicine at the University of Southern California, Los Angeles, California, U.S.A.

3. Department of Radiology, Keck School of Medicine at the University of Southern California, Los Angeles, California, U.S.A.

4. Department of Medicine, Keck School of Medicine at the University of Southern California, Los Angeles, California, U.S.A.

Abstract

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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Ophthalmology,General Medicine,Surgery

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