Identifier |
walsh_2024_s3_c3 |
Title |
The Path of Least Resistance: Over the Nose, Into the Orbit, and Around the Globe |
Creator |
Sahar Noorani; Ali Tejani; Mingyi Chen; Melanie Truong-Le |
Affiliation |
(SN) (AT) (MC) (MT) University of Texas Southwestern Medical Center |
Subject |
Vasculitides; Extraocular Muscles; Vision Loss; Binocular Diplopia; Magnetic Resonance Imaging (MRI) |
Description |
A 45 year-old man presented emergently with progressively worsening blurred vision, oblique binocular diplopia, photophobia, and dyschromatopsia in the left eye for 4 months. He reported swelling around the left eye, unintentional weight loss of 25 pounds, and presence of cervical lymph nodes for several months. Medical history included hypertension, diabetes, asthma with recent exacerbations, and seasonal allergies. He was seen by ophthalmology whose examination of the left eye revealed 3+ rAPD, slight proptosis, -4 abduction deficit, normal color vision via Ishihara color plate testing, and Frisen-like grade 3 disc edema with mild temporal peripapillary atrophy. Contrasted MRI brain & orbits showed enlargement and contrast enhancement of the left lateral rectus muscle, optic nerve sheath, and cavernous sinus. Serum studies were largely unremarkable including TSH, ANCA, ACE, lysozyme, IL2, IL6, MOG/NMO antibodies, thyroid antibodies, immunoglobulins, SPEP, syphilis, Lyme, and TB. ANA was positive with titer 1:320. CSF studies showed normal protein, cell count, and RBCs; elevated glucose (consistent with hyperglycemia); and unremarkable IgG index, ACE, VDRL, and fungal studies. He was started on prednisone 1 mg/kg daily with significant improvement in his ocular symptoms including resolution of blurred vision with acuity of 20/20-2, 1+ rAPD, and -1 abduction and supraduction deficits causing residual diplopia on extreme left gaze. There was improvement of edema around the orbit and optic disc of the left eye. Repeat MRI brain & orbits showed disease improvement. A diagnostic procedure was performed. |
History |
To determine a definitive diagnosis, biopsy of the left lateral rectus muscle was planned. Given the improvement seen on imaging and to optimize biopsy yield, the patient was weaned off steroids. However, he deteriorated almost immediately after cessation of steroids and developed worsening diplopia, blurred vision, proptosis, ophthalmoplegia, and optic disc edema. He also developed an enlarged left-sided lacrimal gland and pre-auricular and cervical lymph nodes. Due to concern for an infiltrative process, he was sent to the ER for expedited work up. Repeat contrasted MRI brain & orbits revealed worsening of his disease process. He underwent biopsy of the left lateral rectus muscle, orbital fat and lacrimal gland, and he was then restarted on prednisone 1 mg/kg. In the interim, CSF cytology was negative for malignancy. Biopsy results showed reactive lymphoid infiltrates with eosinophilia and no evidence of malignancy. It was then noted that while off prednisone, he had peripheral eosinophilia with an absolute eosinophil count of 1.99 (normal 0.00-0.70 x10(9)/L). He was seen by the Allergy and Immunology clinic, who determined he met criteria for eosinophilic granulomatosis with polyangiitis (EGPA).[1,2] He had asthma with recently worsening disease, peripheral eosinophilia >10%, paranasal sinus disease, and extravascular eosinophilia on biopsy. He was ANCA-negative, which can occur in 60-70% of patients with EGPA and is associated with increased cardiac and pulmonary involvement.[2,3] It is not typical for EGPA to present with ocular manifestations, which can be subdivided into idiopathic ocular inflammation and ischemic vasculitis.[3] The patient was started on mepolizumab, an anti-IL5 monoclonal antibody.[4] Presently, his symptoms have nearly resolved, and his neuro- ophthalmic exam shows near-complete resolution of optic disc edema in the left eye, though he has a persistent abduction deficit. He continues mepolizumab and is gradually tapering prednisone. |
Disease/Diagnosis |
Eosinophilic granulomatosis with polyangiitis involving the extraocular muscles |
Date |
2024-03 |
References |
1. Masi, Hunder, Lie, Michel, Bloch, Arend, Calabrese, Edworthy, Fauci, Leavitt. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 33(8), 1094-1100, 1990. 2. Grayson, Ponte, Suppiah, Robson, Craven, Judge, Khalid, Hutchings, Luqmani, Watts, Merkel, DCVAS Study Group. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 81(3), 309-314, 2022. 3. Akella, Schlachter, Black, Barmettler. Ophthalmic eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a systematic review of the literature. Ophthal Plas Reconstr Surg. 35(1), 7-16, 2019. 4. Wechsler, Akuthota, Jayne, Khoury, Klion, Langford, Merkel, Moosig, Specks, Cid, Luqmani, Brown, Mallett, Philipson, Yancey, Steinfeld, Weller, Gleich. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 376, 1921-1932, 2007. |
Language |
eng |
Format |
video/mp4 |
Type |
Image/MovingImage |
Source |
2024 North American Neuro-Ophthalmology Society Annual Meeting |
Relation is Part of |
NANOS Annual Meeting 2024 |
Collection |
Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
Publisher |
North American Neuro-Ophthalmology Society |
Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah |
Rights Management |
Copyright 2024. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
ARK |
ark:/87278/s6pxadk9 |
Setname |
ehsl_novel_fbw |
ID |
2592826 |
Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6pxadk9 |