Focal Pachymeningitis Induced Papilloedema as an Early Manifestation of Relapsing Polychondritis

A 52-year-old male patient presented with longstanding non-specific symptoms of ocular redness and irritation. Clinical examination not only revealed bilateral anterior scleritis but also bilateral optic disc swelling. Additional history taking revealed headaches and tinnitus, both starting around the same time as the eye redness, as well as a prior episode of swelling and redness of both ears. The lumbar puncture opening pressure was 29 cm of cerebrospinal fluid (CSF). There were 11 white blood cells/µl in the CSF. Subsequent magnetic resonance imaging showed focal thickening of the dura mater over the left cerebral convexity, suggestive of focal pachymeningitis. 18F-fluorodeoxyglucose positron emission tomography demonstrated hypermetabolic abnormalities located at the auricles, the nostrils, the anterior part of the eyes, and the dura mater over the left cerebral convexity, suggestive of relapsing polychondritis (RPC). RPC is a rare systemic immune-mediated condition; diagnosis can sometimes be delayed or missed due to insidious disease onset with non-specific symptoms. Nevertheless, sight-threatening or even life-threatening complications may occur. Given the high prevalence of ocular involvement, one should be suspicious when faced with patients with recurrent ocular inflammation. Optic disc swelling is a more uncommon finding, and while different mechanisms have been reported, it has rarely been associated with raised intracranial pressure. Nevertheless, intracranial hypertension arising from inflammation of the CSF and/or surrounding meninges caused by the newly diagnosed RPC was identified as the most likely underlying mechanism for the bilateral optic disc swelling in our patient.