Retrospective analysis of chronic relapsing inflammatory optic neuropathy
ECTRIMS Online Library. Thuringer A. 09/13/19; 278304; P1102
Amanda Thuringer
Amanda Thuringer
Contributions
Abstract

Abstract: P1102

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS Variants

A. Thuringer, S. Lynch

Neurology, University of Kansas Health Systems, Kansas City, KS, United States

Introduction: Optic neuritis is usually considered to be an isolated phenomenon, or associated with multiple sclerosis or neuromyelitis optica. However a chronic, relapsing, idiopathic, and immunotherapy sensitive form has been well-described and termed chronic relapsing inflammatory optic neuropathy (CRION). Early recognition of CRION is important to initiate proper treatment to preserve vision.
Objectives: To provide an updated characterization of patients diagnosed with CRION at our medical center.
Aims: Aim 1: Examine demographics and presenting characteristics of those with CRION. Aim 2: Compare clinical course and response to immunotherapy.
Methods: We identified and reviewed 3,480 patients seen at the University of Kansas Health System from January 2004 to November 2018 with a diagnosis of optic neuritis, neuropathy, or atrophy. Those who met diagnostic criteria for CRION were further analyzed. Data was collected regarding demographics, history of present illness, relevant comorbid conditions, diagnostic evaluations, treatments and visual outcomes.
Results: Twenty patients met diagnostic criteria for CRION. Other inflammatory optic neuropathies identified include multiple sclerosis (n=496), neuromyelitis optica (n=37), paraneoplastic (n=12), and myelin oligodendrocyte glycoprotein (n=4). For CRION patients, average age of onset was 39.3 years old. Ninety-percent were female. At onset, 75% presented with unilateral optic nerve involvement. Average number of relapses per patient was 4.5 over 3.0-year average follow-up duration. Heterogeneity in clinical course was recognized, as two patients were steroid-dependent despite multiple failed steroid-sparing immunotherapies, while others were clinically inactive or did not require immunotherapy (n=6) at last documented follow up. At last follow up, a majority (n=15) were on immunotherapy, which included azathioprine (n=5), mycophenolate (n=4), methotrexate (n=3), prednisone (n=3), leflunomide (n=1), ocrelizumab (n=1), rituximab (n=1), and intravenous immunoglobulin (n=1).
Conclusions: CRION is a relatively uncommon debilitating disease which often requires longterm immunosuppression to preserve vision. Variation in presentation, clinical course, and treatment response is apparent in our cohort. Vigilance is needed to identify CRION patients and treat them appropriately.
Disclosure: Amanda Thuringer, DO: I have received scholarship funding from NMSS and Biogen.
Sharon Lynch, MD: I have received research funding from Novartis, Genzyme, Sanofi, Roche, Mallinckrodt, MedDay, TG Therapeutics, Actelion, Adamas, PCORI, NIH, and the NMSS.

Abstract: P1102

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS Variants

A. Thuringer, S. Lynch

Neurology, University of Kansas Health Systems, Kansas City, KS, United States

Introduction: Optic neuritis is usually considered to be an isolated phenomenon, or associated with multiple sclerosis or neuromyelitis optica. However a chronic, relapsing, idiopathic, and immunotherapy sensitive form has been well-described and termed chronic relapsing inflammatory optic neuropathy (CRION). Early recognition of CRION is important to initiate proper treatment to preserve vision.
Objectives: To provide an updated characterization of patients diagnosed with CRION at our medical center.
Aims: Aim 1: Examine demographics and presenting characteristics of those with CRION. Aim 2: Compare clinical course and response to immunotherapy.
Methods: We identified and reviewed 3,480 patients seen at the University of Kansas Health System from January 2004 to November 2018 with a diagnosis of optic neuritis, neuropathy, or atrophy. Those who met diagnostic criteria for CRION were further analyzed. Data was collected regarding demographics, history of present illness, relevant comorbid conditions, diagnostic evaluations, treatments and visual outcomes.
Results: Twenty patients met diagnostic criteria for CRION. Other inflammatory optic neuropathies identified include multiple sclerosis (n=496), neuromyelitis optica (n=37), paraneoplastic (n=12), and myelin oligodendrocyte glycoprotein (n=4). For CRION patients, average age of onset was 39.3 years old. Ninety-percent were female. At onset, 75% presented with unilateral optic nerve involvement. Average number of relapses per patient was 4.5 over 3.0-year average follow-up duration. Heterogeneity in clinical course was recognized, as two patients were steroid-dependent despite multiple failed steroid-sparing immunotherapies, while others were clinically inactive or did not require immunotherapy (n=6) at last documented follow up. At last follow up, a majority (n=15) were on immunotherapy, which included azathioprine (n=5), mycophenolate (n=4), methotrexate (n=3), prednisone (n=3), leflunomide (n=1), ocrelizumab (n=1), rituximab (n=1), and intravenous immunoglobulin (n=1).
Conclusions: CRION is a relatively uncommon debilitating disease which often requires longterm immunosuppression to preserve vision. Variation in presentation, clinical course, and treatment response is apparent in our cohort. Vigilance is needed to identify CRION patients and treat them appropriately.
Disclosure: Amanda Thuringer, DO: I have received scholarship funding from NMSS and Biogen.
Sharon Lynch, MD: I have received research funding from Novartis, Genzyme, Sanofi, Roche, Mallinckrodt, MedDay, TG Therapeutics, Actelion, Adamas, PCORI, NIH, and the NMSS.

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