An Atypical Presentation of Relapsing Polychondritis Manifesting As Acute Angle Closure

Relapsing polychondritis (RPC) is a rare autoimmune disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. The most commonly affected sites are the auricular pinna, nose, and joints with ocular tissue involvement occurring in up to 50% of patients. The most common ocular manifestations are scleritis, episcleritis, uveitis, and conjunctivitis. Less common ocular issues include keratitis, retinopathy, optic neuropathy, extraocular muscle palsy, and orbital inflammation. Due to the variable clinical presentation and rarity of the disease, the diagnosis of RPC is often delayed or it is misdiagnosed. It is important that ophthalmologists be aware of relapsing polychondritis because ocular symptoms may present as the initial manifestation of the disease.