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Abstract
Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of inflammation in RPC, and a variety of ocular symptoms are observed in approximately half of the patients with RPC. Scleritis/episcleritis, uveitis, and conjunctivitis are common symptoms associated with RPC. Less frequently, keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are also observed. Ocular inflammation could also be the first manifestation of RPC. Although RPC is a potentially fatal and sight-threatening disease, the rarity of the disease and its protean clinical presentation may lead to delayed diagnosis or misdiagnosis. Given the high prevalence of ocular involvement in RPC, to avoid misdiagnosis, physicians should be suspicious of RPC when they see patients with recurrent ocular inflammatory conditions and various systemic symptoms. In this article, we provide a comprehensive review of ocular manifestations associated with RPC.
Highlights
Relapsing polychondritis (RPC) was first described by Jaksch-Wartenhorst [1] in 1923 as a rare systemic immune-mediated disease of unknown etiology characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body [2,3,4]
Peripheral ulcerative keratitis is reportedly a common type of keratitis associated with RPC [10,45,46,47,48], which is similar to other connective tissue diseases, such as rheumatoid arthritis [49]
In 1976, McAdam et al first proposed the diagnostic criteria of RPC [2]: three or more of six clinical features, including auricular chondritis, nonerosive inflammatory polyarthritis, nasal chondritis, respiratory tract chondritis, audio vestibular damage, and ocular inflammation, are needed for diagnosis
Summary
Relapsing polychondritis (RPC) was first described by Jaksch-Wartenhorst [1] in 1923 as a rare systemic immune-mediated disease of unknown etiology characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body [2,3,4]. The rarity and wide spectrum of clinical symptoms and signs of RPC often lead to misdiagnosis or delayed diagnosis [8,9]. Given that RPC is a potentially fatal disease, prompt and correct diagnosis and treatment are essential. The eyeball and ocular adnexa are important target tissues for RPC-induced inflammation, and a wide range of ocular manifestations are observed in patients with RPC (Table 1) [10,11]. We provide an updated, comprehensive review of ocular manifestations in patients with RPC. Retinopathy, retinal vein and artery occlusion, retinal detachment, Retinal vasculitis, retinal pigment epithelium defects, Cystoid macular edema, choroiditis
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