2019 Research Forum

Relapsing Polychondritis Presenting as Optic Neuritis and Lymphocytic Meningitis: A Case Report

David Contreras MD R1, Varun Bali MD R2, Katayoun Sabetian MD, Boa Quynh Huynh MD, Sandra Sofinski MD, Arash Heidari MD

Introduction

Conclusions

Work Up

Lumbar Puncture

Relapsing Polychondritis • Rare disorder affecting 3.5 per 1 million people per year. • Characterized as immune mediated inflammation of predominantly cartilaginous structures • -ears, nose, eyes, and laryngeal tracheobronchial tree. • Most commonly presenting as unilateral or bilateral external ear inflammation. • Other anatomic areas or organs may be involved such as a costal cartilage, eyes, nose, joints, and nervous system. • Diagnosis is often missed or unrecognized for month to years because of intermittent manifestations of symptoms • Blurred vision • Photophobia • Progressive blurred vision over the next 5 days • Described it as “Gray shade over the center of her vision with blurry peripherals” • Denied prior eye trauma or surgeries • Painless progression of vision loss in both eyes • Street signs difficult to see at a distance • Denied flashes of light and floaters • Past medical history – Empyema at age 4 • Surgeries – Surgical drainage of the empyema (1999) • 23 year old college student with no PMH • Sudden onset frontal headache and blurred vision • Radiation to bilateral temporal region

• Entire infectious and immunological work up was negative Inpatient Rheumatology consult • From the clinical presentation of a fully negative infectious workup coupled with vision changes, headaches, bilateral optic neuritis, chorioretinitis, aseptic meningitis, vertigo, hyperacusis, thinning of the pinna and fingers Lead to a diagnosis of relapsing polychondritis. • She was discharged on 60mg of Prednisone daily • Continued improvement of her vision • Improvement of central scotoma • Return of color vision • Rheumatology initiated a prednisone taper over the next 7 months • Prednisone 10mg  5mg • Return of “fogginess of her glass lens” • Visited the Retina Institute and was told she had return of her optic nerve inflammation • Resumed Prednisone 10mg dose • Need to start disease modifying agent of • Hydroxychloroquine vs methotrexate Uniqueness of the case Rare presentation of an already rare disease Diagnosis of relapsing polychondritis was masked by multiple differential diagnosis of • Aseptic meningitis (cocci or viral) • Central serous chorioretinopathy • Leptomeningeal carcinomatosis Only 19 cases can be found in the literature reporting an association of aseptic meningitis in relapsing polychondritis. • Berliner M, Schmidt KL. Polymorphic ocular involvement in relapsing polychondritis: an early, not always recognized symptom. Z Rheumatology; September 1983; 285-7. • Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic findings in relapsing Polychondritis. Ophthalmology May 1986; 93(5):681-9 • Shen K, Yin G, Yang C, Xie Q. Aseptic meningitis in relapsing polychondritis: a case report and literature review. Clinical Rheumatology Jan 2018; 251-255. • Yoo JH, Chodosh J, Dana R. Relapsing polychondritis: systemic and ocular manifestations, differential diagnosis, management, and prognosis. Ophthalmology; July-Sep 2011; 261-9. References

– Opening pressure 150mm H2O – Glucose 58 (40-75) – Protein 46 (15-45)

– WBC 180 (0-5) – RBC count 25

– Lymph 96% – Neutro 3% – Macropha 1% Infectious and Immunological work up

Serum • Cryptococcus Ag screen • Cysticercus IgG AB • West Nile IgG, IgM • Lyme AB screen • Brucella AB • LCM virus AB IgG/IgM • Quantiferon TB Gold • Sed rate 16 • CRP <0.3 • FTA • Syphilis AB • RPR titter • Imunoglob IgM • Imunoglob IgG • HIV Ab/Ag screen • Angiotensin conv enz • ANA screen, IFA

CSF • Cocci serology • VDRL • TB PCR

History

• HSV-1 DNA • HSV-2 DNA • West Nile IgG/IgM • Enterovirus RNA • Cysticercus IgG AB, WB • AFB smear and culture • Lymphocytic Choriomeningitis virus • Brucella screen • Angiotensin converting enzyme • Cryptococcus Ag

• “Sugar Coating” or “Zuckerguss pattern” • Commonly seen in Leptomeningeal Carcinomatosis • DDX: breast or lung carcinoma or melanoma

Hospital Course

• Continued to have blurred vision • Worsening of her headache

• Started on fluconazole 1000mg; aseptic meningitis picture in a person living in the central valley the concern for cocci meningitis was high • Developed tinnitus, vertigo, hyperacusis, and thinning of her pinna without redness. • Vision continued to worsen, including loss of color vision • Lumbar puncture was performed showed worsening WBC 340, RBC 85, lymph 87%, glucose 44, protein 78 • Due to the acute worsening of her visual acuity • High dose pulse steroids were initiated • Vision, vertigo, hyperacusis began to improve with initiation of steroids • Oncology was consulted for the zuckerguss pattern finding on MRI • PET CT was done and review by oncology which showed no evidence of malignancy • After 3 days of pulse steroids, she was started on prednisone 60mg daily Clinical Course

MRI Imaging

Ophthalmology Evaluation • Confrontational visual field test

• Both eyes: full finger counting with central relative scotoma

• Visual acuity

• Right eye: 20/70 • Left eye: 20/200 • Intraocular pressures (normal 12-22) • Right eye: 10 mmHg • Left eye: 12mmHg • Eye exam of the posterior segment

• Both optic nerves were congested with pre-papilledema • Subretinal fluid beneath the maculae and surrounding the optic nerve (DDx Central serous choroidopathy) • No evidence of hemorrhage

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