2019 Research Forum

Applicant & Principal Investigator: David Contreras MD R1 Faculty Sponsor: Arash Heidari MD

Relapsing Polychondritis Presenting as Optic Neuritis and Lymphocytic Meningitis

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

INTRODUCTION Only 19 cases in the literature report an association of aseptic meningitis and relapsing polychondritis.

PURPOSE Highlighted is a case of relapsing polychondritis (RP) in a 23-year-old Hispanic female who is a student at a local university. She presented with a headache of sudden onset that was accompanied with blurry vision. Upon admission, she was urgently evaluated by ophthalmology which revealed severely decreased visual acuity (20/200 right eye and 20/100 left eye), pre-papilledema, central scotoma with a deferential diagnosis of central serous chorioretinopathy. She was immediately referred to the emergency department and was admitted. Lumbar puncture (LP) showed lymphocytic predominant aseptic meningitis picture with 340 WBC and normal glucose and slightly elevated protein. All infectious work up was unrevealing. MRI of the brain showed abnormal meningeal enhancement primarily in the cerebellar vermis region in a sugar coating or Zuckerguss pattern common in leptomeningeal carcinomatosis. She further suffered vertigo, hyperacusis and thinning of the pinna without redness. Due to acute worsening of her visual acuity high dose pulse steroids were initiated. Her vision and headache improved. A rheumatologic history revealed that the patient herself had been evaluated for polyarthralgia at the age of 11 and had her mother has rheumatoid arthritis and spondyloarthropathy. Her follow up LP and MRI brain revealed resolution of pleocytosis and leptomeningeal enhancement. DISCUSSION Relapsing polychondritis is a rare disorder affecting 3.5 per 1 million people per year. It is characterized as immune mediated inflammation of predominantly cartilaginous structures including the ears, nose, eyes, and laryngealtracheobronchial tree. It commonly presents 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. The diagnosis is often missed or unrecognized for month to years because of intermittent manifestations of symptoms. CONCLUSION The elusive diagnosis of RP was masked by multiple differentials including aseptic meningitis, central serous chorioretinopathy and leptomeningeal carcinomatosis. This case is further unique because only 19 cases in the literature report an associated aseptic meningitis in RP patients.

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