Abstract Details

Title Visual Manifestations in the Setting of Perinuclear Antineutrophilic Cytoplasmic Antibody (P-ANCA) Vasculitis

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) P6 - Poster Session 6 (8:00 AM-9:00 AM)

Poster/Presentation
Number 10-003

Objective To highlight the visual manifestations and imaging findings that could be encountered in the setting of P-ANCA/MPO vasculitis

Background Antineutrophil autoantibodies (ANCAs) directed against myeloperoxidase (anti-MPO) are associated with a small vessel vasculitis in which the characteristic feature is a focal necrotizing damage of capillaries, venules and arterioles. It is known to cause optic nerve manifestations as primary pathology or secondary to meningeal inflammation causing raised intracranial pressure¹.

Design/Methods case report of two patients

Results

Case 1:

A 49-year-old woman presented with painless left monocular vision loss. The examination revealed relative afferent pupillary defect in the left eye. Imaging (MRI brain) revealed enhancement of the left optic nerve sheath and diffuse enhancing dural thickening. Spinal fluid analysis revealed Lymphocytic pleocytosis and cytology/flow cytometry was negative for malignancy. Serum labs were positive for MPO-ANCA antibody. Biopsy of meninges revealed multifocal lymphoplasmacytic infiltration consistent with vasculitis. patient was diagnosed with MPO-ANCA- vasculitis and started on steroids. The patient improved clinically and radiographically with steroids later started on Rituximab.

Case 2:

A 62-year-old male presented with headaches and blurred vision. The examination revealed severe bilateral optic nerve head edema with hemorrhages around superior and inferior vessels. MRI of the brain revealed diffuse, enhancing dural thickening. Spinal fluid analysis showed pleocytosis with lymphocytic predominance and opening pressure of 30 cm H2O. Cytology and flow cytometry were negative for malignant cells. Serum labs showed positive MPO-ANCA antibody. Biopsy of meninges revealed fibrous tissue with inflammatory infiltrate. Patient was diagnosed with MPO-ANCA vasculitis due to clinical manifestations and started on steroids which resulted in significant improvement radiologically and clinically with resolution of papilledema and is now on Rituximab.

Conclusions These cases highlight optic nerve involvement by MPO vasculitis, its radiologic features, and need to consider P-ANCA/MPO in the appropriate clinical setting.

Authors/Disclosures
Naga Pradyumna Kothapalli, MD (Stanford University) PRESENTER	Dr. Kothapalli has nothing to disclose.
Heather Moss, MD, PhD, FAAN (Spencer Center for Vision Research at Stanford)	Dr. Moss has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Verana Health. Dr. Moss has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Legal Firms. The institution of Dr. Moss has received research support from NIH. The institution of Dr. Moss has received research support from Research to Prevent Blindness. Dr. Moss has received intellectual property interests from a discovery or technology relating to health care. Dr. Moss has received personal compensation in the range of $0-$499 for serving as a grant review panel with National Institutes of Health. Dr. Moss has a non-compensated relationship as a Board of Directors with North American Neuro-ophthalmology Society that is relevant to AAN interests or activities.
Shannon M. Beres, MD (Stanford Children's Health/Lucille Packard Children's Hospital)	Dr. Beres has nothing to disclose.

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