Ocular Involvement in Relapsing Polychondritis

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.

Autoimmune Disease Classification Based on PubMed Text Mining

“…Relapsing polychondritis is characterized by recurrent inflammation of cartilaginous tissues (collagen type 2) throughout the body [ 30 ]. Figure 2 shows that relapsing polychondritis is associated with scleritis, uveitis, and conjunctivitis, and both relapsing polychondritis and scleritis are associated with autoimmune otorhinolaryngitis [ 31 ].…”

Recurrent Eye and Ear Pain in an Older Patient

“…Nasal chondritis can cause saddle nose deformity . Ocular involvement, which can cause blindness, affects approximately half of patients and commonly presents with scleritis, episcleritis, uveitis, and conjunctivitis …”

“…6 Ocularinvolvement, which can cause blindness, affects approximately half of patients and commonlypresentswithscleritis,episcleritis,uveitis,andconjunctivitis. 7 In 20% to 50% of patients, relapsing polychondritis affects the larynxortracheobronchialtree,whichmaycauselaryngealortrachealstenosis,tracheomalacia,ortracheobronchialobstruction. 2,8 Suddendeath has been reported due to laryngeal or tracheal spasm or acute airway edema.…”

Rare within rare. Necrotising scleritis and peripheral ulcerative keratitis: eye-threatening complications of relapsing polychondritis

“…In-depth characterisation of RP clusters, including their genetic associations (as in the newly described VEXAS syndrome, for instance) will hopefully bring about information regarding early diagnosis, monitoring and best therapy for RP-associated NS and PUK (56,57). phy, computed tomography, magnetic resonance imaging or 18F-fluorodeoxyglucose-positron emission tomography/CT may help identify chondritis in a case of severe NS or PUK (60). RP has a significant disease burden, and standardisation of treatment approaches and prevention of disease-related complications represent another important unmet need in patients with RP (17).…”