Ocular manifestations of relapsing polychondritis: a case series highlighting neuro-ophthalmic involvement

Relapsing polychondritis (RPC) is a rare, chronic inflammatory disorder that affects cartilaginous tissues throughout the body. Although RPC may also be associated with ocular manifestations, comprehensive clinical studies on these findings are limited. This study aimed to characterize the ocular manifestations, clinical features, and treatment outcomes in patients with RPC.

Retrospective observational case series.

We retrospectively reviewed five consecutive patients diagnosed with RPC based on the Damiani and Levine criteria, at the Department of Ophthalmology, Kochi Medical School Hospital, and its affiliated hospitals. Clinical characteristics, ophthalmic findings, treatments, and visual outcomes were analyzed.

Five patients (three men and two women) with a mean age of 65 years were included. Neuro-ophthalmic manifestations were identified in three patients (60%). These manifestations included optic perineuritis and ocular motor dysfunction, with one patient presenting with both. Other ocular manifestations included scleritis, retinopathy, and conjunctivitis in two patients, uveitis, keratitis, and eyelid swelling in one patient. Four patients (80%) were diagnosed with RPC after an ophthalmological evaluation. All patients responded favorably to systemic corticosteroids and immunosuppressive therapy. Ocular motility disorder and visual acuity recovered after treatment in all patients.

RPC may present with diverse neuro-ophthalmic manifestations, including cranial neuropathy and optic perineuritis, in addition to more commonly recognized inflammatory ocular diseases. Therefore, ophthalmologists should consider RPC in the differential diagnosis of ocular inflammatory diseases accompanied by systemic inflammatory manifestations such as auricular chondritis. Early diagnosis and prompt systemic corticosteroid and immunosuppressive therapy may contribute to favorable visual outcomes.